Physical Therapy of Cerebral Palsy

Physical Therapy of Cerebral Palsy

Freeman Miller, MD Co-Director, Cerebral Palsy Program Alfred I. duPont Hospital for Children Nemours Foundation Wilmington, Delaware Physical Therapy of Cerebral Palsy With CD-ROM Illustrations by Erin Browne, CMI

Freeman Miller, MD Co-Director, Cerebral Palsy Program Alfred I. duPont Hospital for Children Nemours Foundation Wilmington, DE 19899 USA Library of Congress Control Number: 2006933715 ISBN-10: 0-387-38303-4 Printed on acid-free paper. ISBN-13: 978-0-387-38303-3 © 2007 Springer Science+Business Media, Inc. All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, Inc., 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adap- tation, computer software, or by similar or dissimilar methodology now known or hereafter developed is forbidden. The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to proprietary rights. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal re- sponsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. 987654321 springer.com

Preface Cerebral palsy is a lifelong condition that affects the individual, family, and immediate community. Therefore, the goal of allowing the individual with cerebral palsy to live life with the least impact of the disability requires com- plex attention to the individual and the family. Furthermore, society needs to be sensitive and to accommodate individuals with disabilities by limiting architectural impediments and providing accessible public transportation and communication. The educational system provides the key means for helping the individual prepare to function in society to his or her maximum ability. In many ways, the medical care system probably has the least signif- icant role in preparing the child with cerebral palsy to function optimally in society. However, the medical care system is the place where parents first learn that their child has developmental issues outside the expected norm. It is almost universally the place where parents also expect the child to be made normal in our modern society. In earlier times, the parents would expect healing to possibly come from the doctor, but also they would place hope for healing in religion. As this belief in spiritual or miraculous healing has de- creased, a significant font of hope has decreased for parents of young chil- dren with disabilities. The text aims to help the child with cerebral palsy to develop into an adult in whom the effects of the disability are managed so that they have the least impact possible on adult function. This intention is in the context of the fact that the magnitude of improvement in the disability that occurs with ideal management of the musculoskeletal system during growth may be only a small improvement. Probably the more significant aspect of good muscu- loskeletal management through childhood is helping the child and family to maintain realistic hope for the successful adult life of the growing child. This aim requires the medical practitioner to get to know the child and family and to communicate in a compassionate way realistic expectations of the child’s function. For many reasons, the greatest difficulty in providing this kind of care is the limited time practitioners have to spend with the individual pa- tient. There is also the sense, especially among orthopedic physicians, that cerebral palsy cannot be cured (cannot make the child function normally), and thus it is a frustrating condition with which to work. The physician must maintain a balance between communicating hopelessness to the patient and family; and feeling the need to do something, usually a heel cord lengthening, because the parents are frustrated that the child is not progressing. All med- ical decisions, including a surgical option, should always consider both the short- and long-term impact. With every decision the medical practitioner should ask, “What will be the impact of this recommendation by the time the child is a mature adult?” This is the most difficult perspective, especially

vi Preface for young practitioners with little experience. This text is intended to pro- vide this insight as much as possible. Another issue is the poor scientific documentation of natural history and treatment response in cerebral palsy, which has become clearer to me in the course of writing this book. With little scientifically based natural history and few long-term studies, much of what is written in this text is expert- based observation. The goal of writing this is not to say that it is absolute fact, but to provide the starting point of gathering information with the hope that others will be stimulated to ask questions and pursue research to prove or disprove the concepts. The research, which is of help in treating children with cerebral palsy, needs to be planned and evaluated with consideration of its long-term im- pact on the child’s growth and development. All treatment should also con- sider the negative impact on the child. As an example, a number of moder- ately good studies have analyzed the impact of wearing ankle orthotics on the young child. Although the orthotics may provide an immediate benefit by improving the child’s gait, there is probably no long-term benefit. Thus, if the child develops a strong sense of opposition to wearing the brace at 10 years of age because of peer pressure, the brace wear cannot be justified on a cost–benefit analysis. It is also important to consider the quality of the scientific evidence, rang- ing from double-blinded protocols to case reports, but it is equally important not to get hung up on this being the final answer. For example, excellent double-blinded studies show that botulinum toxin decreases spasticity and improves gait for a number of months. Therefore, these studies need to be considered in the context of our goal, which is to give the child the maximum possible function at full maturity. Because no evidence currently suggests that botulinum has either a negative or a positive effect on this long-term goal, the family and physician should decide together if botulinum injection has a pos- itive cost–benefit ratio, as its effects will last only for approximately 6 months. In comparison, no double-blind studies show that Achilles tendon lengthen- ings improve gait three or six months after the surgery, and no such studies are needed because the goal of surgery is to make an improvement in gait several years later and to have improvement at maturity. Most important is that surgery create no disability at maturity. From this perspective, it would be much more useful to have a good controlled case series with a 15-year follow-up than a double-blinded study with six months follow-up. This book should stimulate research that will improve the knowledge base which is focused on the long-term outcome of treatments. However, just be- cause the scientific knowledge base is poor does not mean that we should not apply the best knowledge available to current patients. In addition to research, an individual professional can best extend his or her knowledge base through personal experience. This means that the child and family should be followed over time by the same practitioner with good documentation. By far, my best source of information has been the children whom I have followed for 10 to 20 years with videotapes every year or two. Practitioner experience is ex- tremely important for augmenting the relatively poor scientific knowledge base for musculoskeletal treatment. Careful ongoing follow-up is also crucial to providing hope for the families and the individuals with cerebral palsy. How to Use This CD The CD included with this text is opened with a Web browser. Because the data on the CD is coded with XML and JAVA, only browsers released after

Preface vii 2002, such as Netscape 7.0, Explorer 6.0 or Safari, will be fully able to access this data. Some of the text in the book is organized in topics and is displayed in the section entitled “Main.” All references on the CD have the abstract available on the CD by activating the link associated with the reference. Cases can also be activated from these references in the Main section. There is also a section called “Cases,” which lists all the cases by name as listed in the text of the book. Following these cases are short quiz questions, which can be used to test understanding or study the material on line. There is also a section called “Quizzes,” which lists the quizzes by name of the cases. These quizzes can be opened and answered referring to the full case descriptions. The answers from the quizzes will be tabulated to keep a running total of correct answers for each session. After a quiz is accessed, it will also change color to remind the reader that he has already reviewed that quiz. The sec- tion entitled “Decision Trees” is the treatment algorithms, which are present at the end of each chapter in the book. These decision trees are set up so that area of interest is linked to the text in “Main” for further reading. The section called “Search” is an electronic index to search for specific subjects with in the chapter of the section “Main.” Because of space limitations, only individual chapters can be searched at one time. So if you want to search for “crutches,” you first should activate the Durable Medical Goods chapter, and then search. The results of the search allow you to directly link to the area of interest. The section “History” keeps a running history of the areas that have been assessed, so if you want to return to an area you were reading earlier in the session you can open the history and it will allow you to return to that area. The section “About” includes information on the use of the CD and acknowledgments. In summary, the CD includes videos, case study quizzes, and reference abstracts, which are not inclnded in the book. The book includes significant portions of text not included on the CD, sections on rehabilitation techniques, and a surgical atlas. The book and the CD are intended to complement each other but each can also be used alone. Acknowledgments The production of this book and CD was only possible because of an ex- tensive network of support that was available to me. The support of the administration of the Nemours Foundation, especially the support of Roy Proujansky and J. Richard Bowen in giving me time to work on this project was crucial. It was only through the generous support in caring for my pa- tients by my partners and staff, Kirk Dabney, Suken Shah, Peter Gabos, Linda Duffy, and Marilyn Boos, that I was able to dedicate time to writing. I am very grateful for the generous material provided by all the contributors and for the extensive and extremely important role of the feedback given to me by the consultants. In spite of having an extremely busy practice, Kirk Dabney still found time to read all of the Book. With his wide experience, Michael Alexander made an excellent contribution in the editorial support of the section on rehabilitation. The task of writing and editing would have been impossible without the dedicated work of Kim Eissmann, Linda Don- ahue, and Lois Miller. Production of the CD involved a significant amount of detailed editing and HTML coding, most of which was performed by Linda Donahue. To add a personal touch to the cases, a unique name was as- signed by Lois Miller. The CD required a great effort of technical program- ming to make it work intuitively on all computer formats. Tim Niiler pa- tiently persisted with this frustrating task until it all worked. Videos were masked and formatted by Robert DiLorio. Production of the graphics was a

viii Preface major effort in understanding the complex material in which Erin Browne excelled. This production would have been impossible without her dedica- tion to understanding the concepts and bringing them to visual clarity. Thanks to Sarah West for modeling for the graphics. I would also like to thank the staff of Chernow Editorial Services, especially Barbara Chernow. Without the long support through out the evolution of this book by Robert Albano and his staff at Springer, this project would also have been much more difficult. And finally, I am most grateful for the many families and children who have allowed me to learn from them what it is like to live with the many different levels of motor impairments. It is to the families and children that I dedicate this work in the hope that it will lead to improved care and understanding by medical professionals. Freeman Miller, MD

Contents Preface ........................................................................................ v Contributors .............................................................................. xi Section I Cerebral Palsy Management 1 The Child, the Parent, and the Goal .......................................... 3 2 Etiology, Epidemiology, Pathology, and Diagnosis ...................... 27 3 Neurologic Control of the Musculoskeletal System .................... 51 4 Therapy, Education, and Other Treatment Modalities ................ 107 5 Durable Medical Equipment ...................................................... 137 6 Gait ............................................................................................ 207 Section II Rehabilitation Techniques 1 Neurodevelopmental Therapy .................................................... 345 Elizabeth Jeanson 2 Strengthening Exercises .............................................................. 346 Diane Damiano 3 Balance Interventions.................................................................. 348 Betsy Mullan 4 Electrical Stimulation Techniques .............................................. 349 Adam J. Rush 5 Hippotherapy ............................................................................ 350 Stacey Travis 6 Aquatic Therapy ........................................................................ 351 Jesse Hanlon and Mozghan Hines 7 Assistive Devices ........................................................................ 358 Mary Bolton 8 Seating Systems .......................................................................... 361 Denise Peischl, Liz Koczur, and Carrie Strine 9 M.O.V.E.™ (Mobility Opportunities Via Education) Curriculum................................................................ 368 Kristin Capone, Diana Hoopes, Deborah Kiser, and Beth Rolph

x Contents 10 Occupational Therapy Extremity Evaluation.............................. 371 Marilyn Marnie King 11 Intrathecal Baclofen Pumps ........................................................ 391 Maura McManus References .................................................................................. 398 Index .......................................................................................... 403

Contributors Associate Editors Denise Peischl, BSE Beth Rolph, MPT Kirk Dabney, MD Adam J. Rush, MD Michael Alexander, MD Carrie Strine, OTR/L Stacey Travis, MPT Contributors Consultants Mary Bolton, PT Kristin Capone, PT, MEd Steven Bachrach, MD Diane Damiano, PhD John Henley, PhD Jesse Hanlon, BS, COTA Douglas Heusengua, PT Mozghan Hines, LPTA Harry Lawall, CPO Diana Hoopes, PT Stephan T. Lawless, MD Elizabeth Jeanson, PT Gary Michalowski, C-PED Marilyn Marnie King, OTR/L Edward Moran, CPO Deborah Kiser, MS, PT Susan Pressley, MSW Liz Koczur, MPT James Richards, PhD Maura McManus, MD, FAAPMR, FAAP Mary Thoreau, MD Betsy Mullan, PT, PCS Rhonda Walter, MD

SECTION I Cerebral Palsy Management

1 The Child, the Parent, and the Goal Cerebral palsy (CP) is a childhood condition in which there is a motor dis- ability (palsy) caused by a static, nonprogressive lesion in the brain (cerebral). The causative event has to occur in early childhood, usually defined as less than 2 years of age. Children with CP have a condition that is stable and non- progressive; therefore, they are in most ways normal children with special needs. Understanding the medical and anatomic problems in individuals with CP is important; however, always keeping in mind the greater long-term goal, which is similar to that for all normal children, is important as well. The goal for these children, their families, medical care, education, and so- ciety at large is for them to grow and develop to their maximum capabilities so that they may succeed as contributing members of society. This goal is especially important to keep in perspective during the more anatomically detailed concerns discussed in the remainder of this text. How Different Is the Child with CP? When addressing each of the specific anatomic concerns, the significance of these anatomic problems relative to the whole child’s success needs to be kept in the proper context. The problems of children with CP should be evalu- ated in the perspective of normal growth and development similar to any normal children with an illness, such as an ear infection, who need medical treatment. However, keeping the specific problems of children with CP in the proper context is not always easy. The significance of this proper context is somewhat similar to the significance of having a child do spelling homework on Wednesday evening to pass a spelling examination on Thursday. Like- wise, practicing the piano is necessary to succeed in the piano recital. Even though each of these acts is important toward the final goal of having a con- fident, educated, and self-directed young adult who is making a contribution in society, the exact outcome of each event may not be all that important in the overall goal. Often, the success of a minor goal such as doing well on a specific test is less important than a major failure, but the measure of failure or success may be hard to recognize until years later. As with many child- hood events, the long-term effect may be determined more by how the event was handled than by the specific outcome of the event. For children with CP, in addition to all the typical childhood experiences is the experience of their CP treatment. Different children may experience events, such as surgery and ongoing treatment (including physical and occu- pational therapy), very differently. The long-term impact of these events from the children’s perspectives is often either negative or positive, depending on

4 Cerebral Palsy Management their relationship with both therapists and physicians. These children have physical problems, which are the major focus of this text; however, CP affects the whole family and community. These relationships and how the CP af- fects families and communities are discussed in greater detail in this chapter. The process of growing and developing involves many factors. One of the most important factors in children’s long-term success is a family care- taker. Likewise, for children with CP, families may be impacted by the CP as much as the children with the physical problems. It is very important for medical care providers to see the problems related to CP as not only involv- ing the children, but also involving the families. Society is realizing more that the education of normal children works best when the family care providers actively participate. Likewise, providing medical care for children with CP must consider their whole families. The outcome for these children will be determined largely by their families, just as the success of normal children’s education is determined by their families. The importance of family does not provide an excuse for medical care providers or educators to become pes- simistic if they do not perceive the family is doing its part. In this circum- stance, professional care providers still must give as much as possible to each child but recognize their place and limits in the care of these children. Med- ical care providers who fail to recognize their own limits in the ability to pro- vide care often will become overwhelmed by their sense of failure and will burn out quickly. Family Impacts of the Child with CP A healthy liaison should be developed between children with CP, the family unit, and the medical care providers. Cerebral palsy is a condition that varies extremely from very mild motor effects to very severe motor disabilities with many comorbidities. In addition, there are great variations among families. To provide proper care for children with CP, physicians need to have some understanding of the family structure in which the children are living. Be- cause of time pressures, this insight is often difficult to develop. Families vary from young, teenage mothers who may have the support of their families, to single parent families, to families with two wage earners and other children. All the pressures of caring for a child with a disability are added onto the other pressures that families of normal children have. Because most children with CP develop problems in infancy and early childhood, families grow and develop within the context of these disabilities. Often, the father and mother will react differently or come to different levels of acceptance. It is our impression that these different reactions may cause marital stress leading to high levels of divorce, most frequently when the children are 1 to 4 years old. Although this is our impression, there is no clear objective evidence that the divorce rate for these families is higher than in the normal population. Another high time of family stress is during the teenage or young adult years for those individuals with severe motor dis- abilities. Often, as these individuals are growing to full adult size and the parents are aging, it becomes very apparent to the parents that this is not a problem that is going away, nor are these young individuals capable of go- ing off to college and making a life of their own. The response of an individual family varies greatly with the wide vari- ability of severity of CP. Many families develop a stable and very supportive structure for their disabled child. Physicians and other medical care pro- viders may be amazed at how well these families deal with very complex medical problems. For many of these families, however, the medical com-

1. The Child, the Parent, and the Goal 5 plexities have accumulated slowly and are themselves a part of the growth and development phenomena. With multiple medical treatments often pro- vided by many different medical specialists, a high level of stress develops in almost every family. For the medical professional, continuing to be aware of this stress and listening for it during contact with families is important. Families with less education and limited financial resources may do remarkably well, whereas a family with more education and more financial resources may not be able to cope with the stresses of a child with a severe disability. It is extremely dif- ficult to judge which family can manage and which family will develop dif- ficulty, so it is important not to become prejudiced either for or against spe- cific families. Medical care providers should continue to be sensitive to how the family unit is managing to deal with their stresses. Some families will be seen to be doing well and then suddenly will become overwhelmed in the face of other family stress. This stress may be illness in other family members, financial pressures, job changes, marital stress, and, most commonly, the effects of aging on the parents, siblings, and individuals with CP. Care-Providing Community Children with CP develop in supporting communities, which vary with each individual child. There are four general segments of these caring commu- nities, with the family or direct caregivers being the primary relationship. This primary relationship is surrounded by community support services, the medical care system, and the educational system (Figure 1.1). The com- munity support includes many options such as church, Scouting, camping, Figure 1.1. A large and extensive care team surrounds the family with a child who has cerebral palsy. These care providers are roughly organized around the educational system, primary medical care provider, the cerebral palsy specialized medical team, and community support services. Significant over- lap and good communication provide the best resources to the child and the family.

6 Cerebral Palsy Management respite services, and recreational programs. The educational system includes both educational professionals and therapeutic professionals, especially phys- ical and occupational therapists. The focus of this text is to address the med- ical issues, so there will be no specific discussion of these support services, except to remind medical professionals that other services provide crucial roles in the lives of children and their families. The organization of the med- ical care system tends to organize around the general medical care and the specialty care for the problems specific to CP. It is very important for families to have an established general medical care provider, either a pediatrician or family practice physician. Families must be encouraged to maintain regular follow-up with a primary care physi- cian because very few orthopaedists or other specialists have the training or time to provide the full general medical care needs of these children. Stan- dard immunizations and well child care examinations especially will be over- looked. However, most families see their child’s most apparent problem as the visible motor disability and will focus more medical attention on this dis- ability at the risk of overlooking routine well child care. The physician man- aging the motor disability should remind parents of the importance of well child care by inquiring if the child has had a routine physical examination and up-to-date immunizations. A physical or occupational therapist will pro- vide most of the medical professional special care needs related to the CP. The specialty medical care needs are provided in a specialty clinic, usually associated with a children’s hospital. Cerebral Palsy Clinic Another way to organize the management of these well child care needs is with a multidisciplinary clinic in which a primary care pediatrician is pres- ent. The administrative structure for setting up a clinic to care for children with CP is not as well defined as it is for diseases such as spina bifida. Spina bifida, meningomyelocele, or spinal dysfunction clinics are all well-established concepts and are present in most major pediatric hospitals. These clinics, which are set up to manage children with spinal cord dysfunction, have a well-defined multidisciplinary team. This team works very well for these chil- dren because they all have similar multidisciplinary needs ranging from neu- rosurgery to orthopaedics, urology, and rehabilitation. However, this model does not work as well for children with CP because their needs vary greatly. These needs range from a child with hemiplegia who is being monitored for a mild gastrocnemius contracture only to a child who is ventilator dependent with severe osteoporosis, spasticity, seizures, and gastrointestinal problems. It is impossible to have all medical specialists available in a clinic setting, es- pecially in today’s environment where everyone has to account for their time by doing productive work, described mainly as billable time. There are two models currently being used in most pediatric centers for the care of children with CP. One model has a core group of clinicians who see the children, often including an orthopaedist, pediatrician, or physiatrist, social worker, physical therapist, and orthotist. The second model consists of families making separate appointments for each required specialist. The advantage of the first model is that it helps families coordinate their child’s needs. The major disadvantage is that it is costly and not reimbursed by the fragmented American healthcare system. The advantage of the second system is its efficiency to healthcare providers; however, there is often no communi- cation between healthcare providers, and the responsibility of coordinating care from many different specialists thus falls to families.

1. The Child, the Parent, and the Goal 7 From a practical perspective, considering the cost restriction of the healthcare environment, the best system is some blending of the two clinic models. We use this blended model, and it works for many patients with CP and their families. We schedule outpatient clinics where an orthopaedist and pediatrician share the same physical office space; however, each child is given an individual appointment with each physician. If there are only musculo- skeletal concerns, only the orthopaedist is scheduled to see the child. How- ever, if a child also has additional medical needs, the pediatrician is seen before or after the orthopaedic appointment. Orthotics, rehabilitation engi- neering for wheelchair services, nutritionists, social workers, and physical and occupational therapy are available in very close proximity to this out- patient clinic. If a child had a recognized problem before the clinic visit, appointments would have been made to see any of these specialists. How- ever, if the problem is found at the current visit, such as an orthosis that is too small, this child can be sent to the orthotist and be molded on the same day for a new orthotic. This clinic also has a special coordinator to help par- ents schedule appointments with other specialists such as dentistry, gastro- enterology, or neurology. This structure is most efficient for medical care providers; avoids dupli- cation of services, such as having a physical therapist evaluate a child who is getting ongoing community-based therapy; and can potentially provide maximal efficient use of the parents’ time. The main problem arising with this system is that it requires cooperation between many areas in the hos- pital. This model only works if the needed specialists are all working on the same day and are willing to work around each other’s schedules. For exam- ple, holding the CP clinic on a day that the dental clinic is closed or the or- thotist is not available does not work. Although individual appointments are made with specialists, schedules often are not maintained perfectly, so if the orthopaedic appointment is for 10 a.m. but the child is not seen until 11 a.m., the time of the next appointment with a neurologist, all the schedules are af- fected. Making this system work requires flexibility by all involved. One area of efficiency that the medical care system pays little attention to is the parents or caretaker’s time. Most caretakers have to schedule a whole day to take a child to a physician appointment because it means tak- ing the child out of the school, usually driving some distance, seeing the physician, then returning home. This system of actively trying to schedule a number of appointments on the same day allows parents to make use of the whole day, avoiding more days out of work for the parent and out of school for the child. Coordination between team members is accomplished by weekly team meetings where outpatient children with specific needs, along with pending and present in-hospital patients, are discussed. No matter what administra- tive structure is used for the outpatient management of children with CP, because of the diverse population and needs, there are always individuals who will not fit the structure. Therefore, an important aspect of providing medical care to this patient population is to have some flexibility in the de- livery system. Family Care Provider and Professional Care Provider Relationship The specific organizational model for providing care is not as important as the fact that the medical care provided to the child with CP must always be

8 Cerebral Palsy Management provided to the family–child unit. This relationship may be somewhat dif- ferent for educational professionals than for medical care professionals. This discussion focuses primarily on the medical care professional relationship, specifically on the care of the motor disabilities provided by a physician. The first aspect of treating children with CP is ensuring that the families have heard and come to some level of acceptance that their child has a prob- lem called CP, which is permanent and will not go away. Hearing and ac- knowledging a diagnosis is a process that requires families first to come to terms with hearing the words and, second, to internalize these words. This process may take many years, with families initially acknowledging that there is a problem, but still expecting a cure soon. In the initial session with families to discuss this diagnosis, it is important that physicians allow plenty of time to answer all their questions, do not demand that they immediately accept the physicians’ words, and avoid definitive words that bring a sense of hopelessness to families. During this discussion with families, there is little role for the use of absolutist terms like “never,” “will not,” “cannot,” “will die,” or “will never amount to anything.” These terms often strike families as extremely cruel and threaten to remove all their hope, which they desperately need. Having time to answer all a family’s questions and allow- ing them to have their own doubts is important. As the physician relation- ship develops with a family, especially in the context of a clinic for CP, the families will slowly come to their own realization. However, this process of coming to terms with the diagnosis may be impacted by the circumstances and situations surrounding the etiology. Family Response Patterns All families come to terms with their children’s problems in their own way; however, there are several problems that are based on mechanisms surround- ing the inciting event or the time of the diagnosis. In general, most families struggle to understand why this happened to their children and who is at fault. Obstetric difficulties surrounding delivery can be the clear cause of CP. However, many of these birthing problems are probably due to a fetus that was already sick. Nevertheless, the birthing problems often focus the parents on looking for someone to blame, frequently the obstetrician. Some families can come to the point where they can release this need to blame; for others, it may lead to finding a legal solution by way of bringing a legal suit against the individual or organization perceived to be at fault. These legal pursuits are often encouraged by lawyers, and for many families, this only leads to more disappointment when some of the legal efforts are unsuccessful. For families who win legal judgments, there may be some sense of justice; how- ever, the difficulty of caring for a child with a disability continues, and the need to come to terms with why this happened does not disappear by receiv- ing money from a successful lawsuit. Some parents, who have difficulty dealing with why this happened to their child, will be very suspicious of the medical system and will be perceived as being very difficult. There is a tendency for medical care providers, doc- tors, nurses, and therapists to avoid contact with these families, which often leads to more stress because the families feel that they are being avoided. This kind of very suspicious family, especially with underlying unresolved anger related to the initial diagnosis, needs to be kept exceptionally well informed and have frequent contact with the senior attending physician.

1. The Child, the Parent, and the Goal 9 When a child is hospitalized, it is important to have the attending physi- cian meet with the family frequently and always keep them appraised of changes and expected treatment. This level of communication with families sounds very simple; however, we have seen many families who endured a series of terrible events in hospitals, such as oversights or staff failure to rec- ognize an evolving event that the family already pointed out. When these situations are brought up with staff, such as nurses and residents, there is a tendency for the response to be “they brought it on themselves.” This kind of thinking is unacceptable because lack of contact with the senior respon- sible medical staff is usually the main cause. It is important for medical staff to recognize this pattern of behavior in families and respond very consciously by increasing communication and fre- quent contact. Again, the primary responsibility for this contact rests with the senior treating physician, who must display confidence, knowledge, and control of the situation to comfort the family. These families are very per- ceptive of physicians and care providers who do not have experience and con- fidence in dealing with their children’s problems. Often, these families have considerable experience in hospitals and notice when things are overlooked or symptoms are not addressed in an appropriate time (Case 1.1). Dealing with Blame Medical care providers must not get into situations where they inadvertently inflame this need to blame someone for the cause of these children’s CP. When parents give their perception of the history of the inciting event, it should be accepted as such without comment. Medical care providers should not tell parents how terrible the person they blame was or anything else that gives the impression that the CP could have been avoided if only this or that were done. This kind of postmortem evaluation of past medical events helps medical practitioners to learn; however, a detailed dissection of long-gone biomedical events to look for a person to blame seldom helps the families to come to terms with their children’s disabilities. By far, most of these families’ “need to find someone to blame” is a stable enduring part of their lives, and if the treating physician acknowledges this need and focuses their concerns on the children’s current care and situation, the blame issue tends to fall to the background. There is no need for the orthopaedic physician caring for these children’s motor disabilities to get an extensive history of the birth and delivery directed at understanding the etiology of the CP from the families, so long as the diagnosis of CP is appropriate. Instead, the families’ mental energies should be directed at the goal, which is to help their children be all they can be, given the current circumstances. However, trying to convince the parents that they have to give up looking for a cause or a person to blame is also futile. If the parents are totally immobilized and cannot move forward, arranging psycho- therapy may be worthwhile; however, most parents will perceive this as an- other attempt to sweep away the problem of who is responsible. Another common scenario for the diagnosis of CP is when a parent or grandparent recognizes some slow development in a child. This child was then taken to see the family doctor or pediatrician who reassured the family that they were overreacting. Often, these families end up going to their primary care provider two, three, or four times to hear the same response, that is, that they are just overreacting. The child is a little slow, but there is nothing to worry about. These families often want to lay the blame for the CP upon the

10 Cerebral Palsy Management Case 1.1 Susan Susan was born after a normal pregnancy and delivery at to be broken; however, she was no longer dependent on term and was discharged home from the hospital as a her shunt so shunt repair was not performed. normal newborn. At 3 weeks of age, her grandmother thought that her head looked abnormal, and Susan was By age 13 years, she developed more lethargy and a taken to a pediatrician where a workup revealed hydro- shunt revision was recommended. During this shunt re- cephalus. A shunt was placed at 4 weeks of age, followed vision, she had severe complications including an infection by some complications. After this time, she was noted by that required the shunt to be externalized. The external her parents and grandmother to be less strong and less drainage was not controlled carefully enough and, as a interactive. However, she did well, and by age 3 years was consequence, the ventricles collapsed, causing intracranial crawling, rolling, and talking. At age 3 years, she devel- bleeding. This episode caused substantial neurologic func- oped severe seizures and was hospitalized. During this tional loss, so she was now less able to interact socially hospitalization, she had a rather severe overdose of anti- with her parents on top of her very severe spastic quad- seizure medication along with other subsequent compli- riplegic pattern motor disability. In addition, her seizures cations and lost the ability to crawl, roll, and talk. Her increased substantially. This episode made her parents parents started patterning therapy when she did not rap- extremely anxious about medical treatment, especially idly regain these functions. She also started to develop about the fear of developing complications and having increased spasticity and had more trouble with her trunk functional loss. control. Shortly after the shunt problems, she was noted on By age 6 years, Susan had an adductor lengthening routine medical examination to have a retinal detachment and was developing scoliosis. She was started in a body requiring surgery. This surgery occurred without any jacket to help control her scoliosis, and by age 8 years, complications. She continued to have problems with her she had a painful dislocated hip. After the family searched seizures, and her parents were anxious to have control of for several different opinions, they elected to go ahead the seizures, while at the same time to allow her to regain and have the hip reconstructed. Because Susan had sub- some of her alertness and contact with her parents, which stantial complications with loss of neurologic function on they much enjoyed. several previous admissions, her parents were perceived as being extremely anxious during the hospitalization. This family was often perceived by nurses and house The operative procedure and the recovery phase of the staff as being exceedingly difficult to deal with because hip reconstruction went very well and the family was very they were so anxious and always wanted to observe and gracious. understand specific treatments and know exactly which medications were being administered. This family was By age 9 years, she needed to have additional soft- extremely dedicated to the care of their daughter, and the tissue lengthenings of her right shoulder for a painful dis- anxieties that they expressed were very understandable location as well as for progressive varus deformity of the considering their history. Often, medical care providers, feet. The family was less anxious during this procedure especially physicians and nurses, were not aware of this than they had been with the prior procedure because they history and therefore did not understand the parents’ were more comfortable with the staff. anxieties. This anxiety tends to make nursing staff and medical staff try to avoid the parents, which just greatly By age 12, the scoliosis had progressed substantially, increases their anxiety level. These parents had more than requiring a posterior spinal fusion. The family was very one hospitalization per year on average with their daugh- anxious about this very large procedure. Their anxiety ter and were very aware of what her proper medical man- was perceived by some staff as being overreactive; how- agement should be. They were very astute in picking up ever, considering the history of their experience with past inexperience in both the nursing and medical staff and medical treatment, we felt it was appropriate. At the time would become much more anxious when they sensed this of the posterior spinal fusion, the shunt tubing was noted inexperience or discomfort in dealing with their daughter.

1. The Child, the Parent, and the Goal 11 physician, believing that this delayed diagnosis is why the child currently is so severe. There is almost no circumstance where a delayed diagnosis will be of any significance. It is important for these parents to have their concerns about the delayed diagnosis acknowledged, but then they must be reassured that this delay did not, in any way, cause their child to have a greater sever- ity of CP. Some of these families will have difficulty developing other trust- ing relationships with physicians and may call, especially initially, for many minor concerns until confidence in their physician is developed. Sometimes CP is the result of an accident or event in childhood, such as a toddler with a near drowning, or a child with a closed head injury from a motor vehicle accident in which the parent was the driver. In these situations, the parents often feel a substantial amount of blame for causing their child’s disability. This self-blame and guilt may be even more difficult for a parent to come to terms with than blame focused outward. One response to the inwardly focused blame is to search for extraordinary cures, demand more therapy, or get more devices. This behavior seems to be one of “making it up to the child.” It is helpful to reassure the family that things besides more therapy or more devices, such as maximizing the child’s educational ability, will help the child. Giving and Dealing with Prognosis Another experience frequently reported by parents whose children were in neonatal nurseries is the comment that the children probably will not sur- vive, and, if they do, will be vegetables. This comment has been reported to us by parents of children who end up with hemiplegia as well as children with quadriplegia. We believe this comment stems from the great difficulty of making a specific prognosis of outcome in the neonatal period. Also, some physicians tell families the worst possible outcome, believing that when the children do better, the families will be grateful for their good luck. However, this explanation almost never has the intended outcome, and much more commonly the families perceive these comments as the physician being in- competent or deceitful. Often, these families will interpret attempts by later physicians to discuss prognosis or expected results of surgery as being too pessimistic. For these families, it is important to be as realistic as possible; however, their optimism may cause some disappointment as their expecta- tions of greater outcomes are not realized. Generally, these families do come to appropriate expectations, but continue to have some negative feelings about their neonatal experience. An important aspect of giving prognosis or information that is requested by families is to always acknowledge that it is imperfect. Requests to know if a child will walk or sit should be answered as honestly as possible, always avoiding absolutist terms such as “never,” “cannot,” or “will not.” Giving the Diagnosis Another common problem surrounding diagnosis of children with CP is fail- ure to give the parents a diagnosis. A common example of this is a mother of a 5-year-old who is unable to sit and brings the child to see the ortho- paedist to find out why the child cannot walk. The history reveals a normal pregnancy and delivery; however, by age 12 months, the child was not sit- ting, so the mother starting going to doctors to find out what was wrong with the child. She has seen three neurologists and a geneticist, has had skin

12 Cerebral Palsy Management biopsies, muscle biopsies, computed tomography (CT) scan, magnetic reso- nance imaging (MRI) scan, and many blood tests, but everything is normal. The mother hears from these doctors that they can find nothing wrong with her child; however, what the doctors probably told the mother is that the medical tests are normal and they do not know what caused the child’s cur- rent disability. Families need to be told what is wrong with their child. This type of family is easily helped by explaining that the child has CP. Physicians should clearly explain that even though they do not understand why the child has CP, it is the diagnosis, which they know exactly how to treat. Taking time and providing information to these families will stop the endless and futile search for “why” and allow them to focus on caring for and treating their children. This situation is caused almost entirely by physicians not being clear in communication with parents and the particular aversion by some physicians to giving a diagnosis of CP. This aversion is very similar to want- ing to avoid telling a patient that she has cancer, and therefore telling her that she has a nonbenign growth whose cause cannot be explained. In this way, CP is like cancer in that a physician often cannot determine the etiol- ogy; however, the treatment options are well defined and should be started immediately. Medical Therapeutic Relationship to Child and Family There are many different types of therapeutic relationships that work for families and their children; however, there are some patterns that work bet- ter than others. These patterns each have their risks and benefits as well. The major therapeutic relationships in the treatment of motor problems of chil- dren with CP include the parents, the physical therapists, and the physicians. The parents will spend the most time with their children and will know them best. Often, the parents recognize developmental gains and day-to-day vari- ability in their child’s function first. Physical therapists will spend the most therapeutic time during treatment with children and will bring the expe- rience of similar children. This in-depth experience with similar children al- lows therapists to help parents understand the expected changes as well as teach parents and children how to maximize their function. The orthopaedist treating the motor disability will have the least experience with an individual child, but will have the broadest experience with many children to under- stand the expectations of what will occur. The physician’s experience with each child, however, will be much more superficial and the physician depends on the parents’ and therapists’ observations of the children’s function over time and the variability of function during the day. Recognizing these indi- vidual strengths will allow the parents’, therapists’, and orthopaedists’ per- ception of individual children to be combined to make the best therapeutic judgment. The Physical Therapist Relationship The role of the primary treating physical therapist, especially for the young child between the ages of 1 and 5 years, will incorporate the typical role that the grandmother and the general pediatrician play for normal children. In addition, the therapist fulfilling this role must have knowledge and experience

1. The Child, the Parent, and the Goal 13 in dealing with children with CP. This role model involves time spent teach- ing the parents how to handle and do exercises with their child. This role also involves helping the parents sort out different physician recommen- dations, encouraging the parents, and showing and reminding parents of the positive signs of progress in the child’s development. When this role works well, it is the best therapeutic relationship a family has. The positive aspects of this role are providing the parents with insight and expectations of their child, reassuring the family that they are providing excellent care, and being readily available to answer the family’s questions. The “grandmothering” role of the therapist has associated risks. One of the greatest risks in our current, very unstable medical environment is that a change in funding or insurance coverage may abruptly end the relation- ship. An abrupt change can be very traumatic to a family. The therapist must be careful not to be overly demanding of the family, but to help the family find what works for them. Occasionally, a therapist may be fixated on a spe- cific treatment program and believe that it is best for the child; however, the parents may not be in a situation to follow through with all this treatment. The parents feel guilty, and the therapist may try to use this guilt to get them to do more. The physical therapist in this role as a therapeutic “grandmother” can help parents sort out what medical care and choices are available. The ther- apist can help parents by attending physician appointments and making the parent ask the right questions, which is often not possible because of fund- ing restrictions. The physical therapist must not give specific medical advice beyond helping parents get the correct information. Therapists with exten- sive experience should recognize that they have great, detailed, and deep experience with a few children and that generalizing from the experience of one child is dangerous. We have heard therapists tell parents on many occa- sions that their child should never have a certain operation because the therapist once saw a child who did poorly with that surgery. This type of ad- vice is inappropriate because one child’s experience may have been a rare complication of the operation. Also, there are many different ways of doing surgery. This would be like telling someone to never get in a car again after seeing a car accident. A more appropriate response to the family would be giving them questions to ask the doctor specifically about the circumstance with which the therapist is concerned and has experience. Another physical therapist therapeutic relationship pattern is the purely clinical relationship in which the therapist thinks the family is incompetent, unreliable, or irresponsible and only wants to deal with the child. Almost invariably, this same therapist next will complain that the family and child never do the home exercise program or that the child is not brought to ther- apy regularly. This relationship may work for a school-based therapist or a therapist doing inpatient therapy, but it leads to great frustration for both the therapist and family when it is applied to an outpatient-based, ongoing developmental therapy. In this environment, the therapist must try to under- stand and work within the family’s available resources. The Physician Relationship Families of children with CP often have a series of physician relationships and tend to choose the physician with whom they are comfortable, who re- sponds to their needs, and who is able to help them with their child’s prob- lems. As pediatric orthopaedists, many of our patients will report to their schools and emergency rooms that we are their child’s doctors. We strongly

14 Cerebral Palsy Management encourage families to have family doctors or general pediatricians to care for well child care needs and minor illnesses. With the changing healthcare pay- ers, some families have changed family doctors every year or two and the physician who cares primarily for the musculoskeletal disabilities of a child often becomes defined as the child’s doctor. The musculoskeletal problems of CP are well known and are relatively predictable; therefore, a major part of the treatment is educating the family of what to expect. For example, a nonambulatory 2-year-old child who is very spastic has a high risk of developing spastic hip disease. This risk needs to be explained to parents so they know that routine follow-up is important and that, if spastic hip disease is found, there is a specific treatment program. At each visit, this plan is reviewed again. Diligent attention to this individ- ual education process gives parents a sense of confidence about the future and helps prevent the development of a nihilistic family approach that noth- ing can be done for their child. Because families usually start to see the CP doctor when the children are about age 2 years, and in our clinic stay until age 21 years, a long-term relationship is developed. Keeping a healthy therapeutic relationship, under- standing and taking into consideration the family’s strengths and limits, is important. In addition to helping the family understand what to expect with their child, continuing to support the family as much as possible is very im- portant. One easy way to give the family positive feedback is to focus on the positive things that the child has accomplished, such as better physical func- tioning, good grades, good behavior, gaining weight, growing taller, and being nicely dressed. There is a tendency for parents to only hear negative things from doctors, such as a catalog of all the things the child cannot do. Another aspect of the therapeutic relationship is recognizing that this is not a family relationship. Many of our patients are very happy to see us and we enjoy seeing them; however, as they grow and develop, their doctor should be a positive influence but not their main adult role model. These chil- dren should not be seen more than every 6 months unless there is an active treatment program such as one following surgery. One goal of the medical treatment of these children should be to have as little direct impact as pos- sible on their normal lives so that they grow up having experiences similar to normal children. To this end, medical intervention should be limited as much as possible and should be episodic so that it more closely mimics nor- mal childhood medical experiences, such as fractures or tonsillitis. Frequent trips to a doctor’s office or to a clinic are also very time consuming for fam- ilies. There are almost no musculoskeletal problems that need to be moni- tored more than every 6 months. Recognizing the strengths and weaknesses in families and trying to work within their limits to provide medical care for children with motor disabilities is important. The medical system is limited to working within the confines of what the families and school environment can provide, especially for chil- dren with severe physical disabilities. The state social service protection agencies seldom get involved or are very helpful to families, except in rare dire circumstances. When the Doctor–Family Relationship Is Not Working Medical care providers need to understand that personalities are such that one individual can never meet everyone’s needs. This does not mean that as soon as the doctor therapist family relationship becomes difficult, it is not

1. The Child, the Parent, and the Goal 15 working. At this time, the relationship needs to be discussed and the physi- cian or therapist should be open about giving the family permission to go to another doctor or therapist. Some families will just leave without saying any- thing and others will feel guilty about wanting to leave. Physicians and ther- apists must be honest with themselves because this situation tends to make them feel like a failure. There may be a combined sense of relief that the fam- ily left and a sense of failure and anger that the family does not trust their physician or therapist. These are normal feelings that the physician or thera- pist should acknowledge and not place blame on themselves or the family. When the Family Chooses Medical Treatment Against the Physician’s Advice Families may seek a second opinion for a specific treatment recommenda- tion. This desire to get a second opinion should not be seen by the primary treating physician as a lack of faith or confidence. The family may require a second opinion for insurance purposes or, for many families, they just want to make sure they are getting the correct treatment. Usually, getting a second opinion should be viewed as a very prudent move on the family’s part and should be encouraged. Families should be given all the records and support that are needed for them to get a meaningful second opinion. If this second opinion is similar to that given by the primary physician, the family is often greatly comforted in moving ahead. However, there is still variability in med- ical treatment for children with CP, so depending on the family’s choice of opinions, the recommendations may be slightly to diametrically opposed. In a circumstance where the recommendation of another physician dif- fers significantly, the primary physician must be clear with the family and place the second opinion in the perspective of their recommendation. Some- times the words used may sound very different, but the recommendations are very similar. In other circumstances, the recommendation may be dia- metrically opposed and the primary physician must recognize this and ex- plain to the family the reasons for their recommendation. When recom- mendations are diametrically opposed, clear documentation, including the discussions concerning the other opinion, is especially important. This situ- ation has a high risk for disappointment. Often, families have great difficulty in choosing between divergent opinions, even when one opinion is based on published scientific data and the other opinion is completely lacking in any scientific basis (Cases 1.2, 1.3). Therefore, a family may base their decision on other family contacts, a therapist’s recommendations, or the personality of the physician. Physicians must understand that it is the family’s responsibility and power to make these choices; therefore, with rare exception, no matter how medically wrong the physician believes these decisions are, the family must be given the right to choose. Only in rare, directly life-threatening circum- stances will a child protective service agency even consider getting involved, and then this involvement is usually very temporary. With a long and chronic condition such as CP, temporary intervention by a child protective agency generally is of no use in interacting with families. With clear documentation of the recommendations, the physician must let the family proceed as they choose; however, we always tell them that we would be happy to see them back at any time. When they undergo treatment against their primary physi- cian’s advice and return, usually after several years, the physician should not make the previous situation a conflict. The family usually feels guilty and may not want to discuss past events. Occasionally, they will come back and

16 Cerebral Palsy Management Case 1.2 Judy Judy was born premature as one of twins and weighed parents were told simply that reconstruction was no longer 1300 g. She was in the neonatal nursery for many weeks. possible, and she now required some palliative treatment. Her development was noted to be significantly delayed Her parents were assured that good treatment was avail- early on, and her CP was recognized within the first 2 years able to get rid of her pain; they were informed of the treat- of her life. By school age, Judy was not able to walk, but ment options, and it was strongly recommended that these was able to do some speaking, and there was concern options be pursued. Surgery was scheduled emergently and about her educational ability. At age 7 years, she was seen was completely successful in alleviating her pain. by a developmental pediatrician for an educational as- sessment. This pediatrician thought that she had excellent This is an example of a family who for unknown rea- cognitive ability, but also noted that she was developing sons chooses alternative medical treatments instead of significant contractures, and recommended follow-up with well-recognized appropriate medical treatment. This type a pediatric orthopaedist. However, she was not seen by a of behavior may be very difficult for a physician to accept. pediatric orthopaedist until age 10 years, when she started This family only saw us once when their daughter was 10 to develop some pain in the right hip. At this point, she was years old, and then did not come back for more treat- in a regular school and was complaining of pain in the hip ment. In these situations a physician can only make the during the school day. An evaluation demonstrated a recommendations, but cannot force the families to follow completely dislocated right hip and severe subluxation of through with treatment. This girl clearly would have been the left hip; however, this hip was an excellent candidate much better served by a reconstruction at age 10 years; for reconstruction because, at age 10 years, she had sub- however, the family had complete control. This family’s stantial growth remaining. Hip reconstruction was recom- choice of treatment was not inappropriate enough legally mended to the family and details were given. For reasons whereby the physician would have gained anything by re- that were never quite clear, this family pursued many other porting the family to child protective services or making options in trying to deal with their daughter’s painful dis- any other efforts to try to force them to have treatment. located hip and eventually decided on a treatment that There are many different types of alternative medical they had located through unknown sources, which con- treatments that families may pursue, some of them per- sisted of having a spinal cord stimulator implanted in her formed by a physician, such as spinal cord stimulators, spinal cord. In addition to the spinal cord stimulator, other which provide absolutely no benefit to this kind of spas- alternative medicine treatments were pursued. The hip ticity or pain. There is nothing that the primary caring pain would get better intermittently and then would flare physician can do except try to persuade the family and up, requiring her to be in bed for several days. By 14 years then accept their decisions. However, it is very important of age Judy had periods of relative comfort between bouts to always leave the family the option of coming back of severe pain, until age 15 when the pain became more when they are ready and then provide appropriate treat- constant and severe. By age 15 years, as she entered high ment, as was done in this situation. school with normal cognitive and educational achieve- ments, the pain got so severe that she could no longer sit Six weeks after this girl’s surgery, at which point all her during the school day. At this point, her parents kept her hip pain was gone, the family noted that she was having home in bed and gave her a variety of different pain med- difficulty sitting because of her scoliosis. They were now ications. She was out of school for 1 year, spending most very keen on moving ahead and having the scoliosis cor- of her time in bed, when her parents finally came back rected. This is a circumstance where although the family with a request to have her hip reconstructed because they feels extremely guilty and are often very hesitant to return now perceived she could no longer deal with the pain. because of fear that the physician will be angry with them, once the appropriate treatment has been performed and At this point, except for getting a brief history, her is successful, the family will become very committed to continuing with appropriate medical care.

1. The Child, the Parent, and the Goal 17 Case 1.3 Rhonda Rhonda was born following a normal pregnancy and that this wound opening was not uncommon, especially normal delivery. She was perceived to be normal until after having been extremely edematous, and the mild 18 months of age when her development was noted to wound drainage was not a concern. be substantially slow and a full evaluation demonstrated an infantile cytomegalovirus (CMV) infection. She con- By postoperative day 17, this wound drainage was tinued to make progress and by age 3 years had started not decreasing and instead was increasing. The patient walking independently and was speaking. She had low was still afebrile, was continuing to make good progress muscle tone with some difficulties with balance. She was with her respiratory status, and was able to be up walk- doing well in a special education class environment until ing in physical therapy. However, based on the amount of age 9 years, when she had sudden complete loss of hear- drainage and the appearance of the wound, it was pos- ing in both ears. An evaluation demonstrated that this sible that this could be a deep wound infection. The hearing loss was in response to the CMV infection. By family was told that the wound did not look good, and age 13 years, she had developed severe scoliosis that that if after 2 more days the drainage did not substantially was making her ambulation difficult. At this point she decrease, a more vigorous exploration would be done. was quite healthy, and although she had not regained any On postoperative day 19, the drainage increased slightly; hearing, she was a full community ambulator. The poste- therefore, a more detailed digital inspection, trying to rior spinal fusion was performed without difficulty, and determine the depths of the wound, was undertaken. The the family was told that based on her excellent general deep fascia was noted to be open at the far superior as- health, a fairly quick recovery was anticipated, with her pect of the wound, and the family was informed that this being ready to leave the hospital in approximately 7 days. was a deep wound infection. The girl needed to be re- turned to the operating room, and the wound surgically However, in the intensive care unit (ICU), on the first cleaned out, then treated with open packing and dressing day following surgery she became quite hypotensive, re- changes. At this time the family was told that she would quiring a substantial bolus of fluid as well as a dopamine now be in the hospital for an additional 4 weeks on intra- for blood pressure support. Blood pressure support was venous antibiotics and wound dressing changes, followed required for 5 days, and she then developed respiratory with probably 2 weeks of home intravenous antibiotics. problems and was on ventilator support for 5 days. Fol- The family was already very anxious about all the com- lowing extubation, she continued to have pulmonary plications in the ICU, and now the deep would infection problems needing positive pressure respiratory support at was another major setback. However, after the parents night. In the meantime, she also developed a mild pneu- went home and discussed the significance of this new monia requiring antibiotic treatment. Instead of being dis- problem with an understanding of the exact timetable charged from the hospital in 7 days, she was discharged that was required, they were able to make family plans. from the ICU to the floor 13 days postoperatively. They came back to the hospital the following day and had more discussions concerning details about the planned During this time, the family became anxious because treatment. After making plans with the specific informa- it was medically difficult to make specific predictions tion they were given, they shared that they had made about what to expect. The family was kept informed and, arrangements for their other children and were comfort- overall, they were able to relax as slow progress was made able and relaxed with the plan. They were prepared for in the ICU. Each day, the family saw that she was stable the 4 weeks, and the remainder of the treatment was very or slightly better. Gains were made, such as discontinu- uneventful. ation of the dopamine for her blood pressure support, then discontinuation of the ventilator. This progress was This case demonstrates how important it is to keep the followed by needing fewer respiratory treatments as her family well informed as complications are occurring. To pulmonary status gradually improved. Being able to see give the family the information, the physician has to rec- these gains, although slow, gave the family hope and ognize the complication and develop a clear treatment understanding that things were progressively improving. plan. There is a tendency, especially in situations where there have been multiple complications and the family By postoperative day 10, she had developed some is very anxious, for the physician to not want to give the superficial wound separation and very minimal drainage; family more bad news. Ignoring problems like deep wound however, she was afebrile because she was being treated infections will not make them go away, and the problem by antibiotics for her pneumonia. The family was informed

18 Cerebral Palsy Management will continue to be frustrating. When a clear treatment of their child’s treatment in any way. In this specific case, protocol with the expected outcome is outlined, and the it was equally important to reassure the family that the family is informed that although this is a substantial set- spine fusion was successful in spite of the current prob- back, it should not compromise the long-term outcome lem and that the rod did not need to be removed. blame the physician for the problems because they have transferred the blame for the recommendation (Case 1.4). Nothing will be gained by bringing up these past problems with the family, and the focus should be to move on with the problems at hand as they present themselves. Recommending Surgery For children who have had regular appropriate medical care, the need for specific orthopaedic procedures is usually anticipated over 1 to 2 years, and as a consequence is not a surprising recommendation. We prefer to have these discussions in the presence of the child. For young children, there is no sense that something is being hidden from them. Children in middle child- hood and young adulthood can take in as much as possible, allowing us, as their physicians, to directly address their concerns as well. For younger chil- dren, those under age 8 years, their main concern is that they will be left alone. We reassure them that we make a major effort to allow the parents to stay with them during preinduction in the surgical suite and again in the recovery room. We also reassure children that their parents will be with them throughout the whole hospitalization. As children get older, especially at adolescence, there is often an adult type of concern about not waking up from anesthesia or having other severe complications leading to death. These individuals may have great anxiety, but have few of the adult coping skills that allow the rationality to say that this surgery is done every day and peo- ple do wake up. Some of these adolescents need a great deal of reassurance, most of which should be directed at trying to get them to use adult rational coping skills. If adolescents are having problems with sleeping or anxiety attacks as the surgery date approaches, treating them with an antianxiety or sedative agent is very helpful. Some adolescents and young adults with mental retardation develop substantial agitation over surgery. Parents of such children are usually very aware of this tendency and may wish to not tell them about having surgery until the day before or the day of surgery. Although this is a reasonable practice for individuals with severe mental retardation who are not able to cognitively process the planned surgery, approaching children who are cog- nitively able to process the event in this way is only going to make them dis- trustful of their parents and doctors. In preparing children and families for surgery, it is important to discuss the expected outcome of the surgery with them. Part of this discussion must focus on what will not happen, specifically that their child will still have CP after the surgery. If the goal is to prevent or treat hip dislocation, showing radiographs to the families helps them understand the plan. They also need to be told what to expect of the procedure from a functional perspective, such as “Will the child still be able to stand? Will the child be able to roll? Will the child’s sitting be affected? Will the child’s walking ability be affected?” For children in whom the surgery is expected to improve walking, showing families videotapes of similar children before and after surgery helps them get a perception of what level of improvement is anticipated.

1. The Child, the Parent, and the Goal 19 Case 1.4 Patricia Patricia was born at 35 weeks weighing 2250 g. She had ter having the surgery, and she stopped blaming herself a relatively normal postnatal course except that she was and the physicians because she understood that every- noted to be very good and slept a lot, even requiring body was trying to do what they thought was best with awakening occasionally to eat. However, by 19 months the knowledge they had available at the time. The mother of age, she had significantly decreased tone in her lower was encouraged to focus forward because, following extremities and trunk, but had increased tone in her right dorsal rhizotomy, some of the spasticity does return and upper extremity with some spasticity and was diagnosed her daughter probably would slowly regain some of the as a right hemiplegic pattern CP. By age 4 years, she was lost function. The functional loss was specifically identified able to sit but had very spastic lower extremities, which as the inability for independent stance, for good assisted caused scissoring and equinus when she was standing. transfers, and for household ambulation while being held She was able to sit on a tricycle and pedal. At this time, by her hands. the parents first heard about dorsal rhizotomies and were very interested in pursuing this method to decrease the Over the next 3 years, some tone did return and this spasticity. By age 5 years, she was walking handheld, but girl was able to do some minimal standing transfers; scissoring substantially, and the parents were pursuing however, she has become very heavy, making it difficult various opinions concerning the dorsal rhizotomy. By for her and her family. She underwent reconstructive sur- age 6 years, the parents had gotten a recommendation to gery of her right upper extremity, which improved her use a transcutaneous nerve stimulator on the upper right, ability to use the right extremity to hold on and assist very spastic extremity. A course of this stimulation was with transfers. Seven years after the dorsal rhizotomy, undertaken even though the child objected because of the she developed a severe kyphosis at the site of the rhizo- discomfort, but the parents persisted for several months tomy that required a posterior spinal fusion. This devel- until it was clear that there was no benefit. opment caused her parents some renewed agitation about their daughter having undergone a procedure that they At age 7 years she was able to stand but could not do still felt was very detrimental. This combination of the independent transfers, although she was doing standing family struggling to deal with their daughter’s disability transfers with considerable scissoring. She was not able to as she is becoming full adult size, and trying to find past walk independently without someone guarding her. The blame for the cause of some of the disability, has made it parents continued to get various conflicting opinions on somewhat difficult for the girl to come to terms with her the merit of a dorsal rhizotomy from several dorsal rhi- own disability. zotomy evaluation programs. Finally the family decided to have the child undergo a dorsal rhizotomy at age 7 years. After the posterior spinal fusion, she developed a sub- After 1 year of intense rehabilitation, the mother was very stantial depression and anxiety syndrome with a period of depressed and angry with herself and with the physicians. pain, difficulty with sleeping, and poor appetite. Initially, After an extensive discussion, the mother volunteered that she was started on amitriptyline to help with the poor she was blaming herself and also the physicians, both those diet and sleep. This medication helped by substantially who recommended for and against the procedure, for her improving her diet; however, she continued with signifi- daughter having undergone a dorsal rhizotomy. She be- cant amounts of anxiety and the amitriptyline had to be lieved the rhizotomy caused her daughter to lose function increased over a 2- to 3-month period instead of being in spite of an extremely intense amount of physical ther- decreased. She was referred for a psychiatric consultation apy work and stress over the year following the surgery. for better pharmacologic management of her depression and anxiety. The improved pharmacologic management, After further discussion, the mother was encouraged as well as some counseling with the parents, has greatly and began to see this experience as an attempt by herself assisted this young woman in making the transition to and her husband to choose what was right for their daugh- young adulthood. ter. The mother was slowly able to acknowledge how dif- ficult it is for a family to make decisions when there are This case is an example of parents who try very hard varying medical opinions about a procedure, especially a to find the latest and best treatment, and after extensive new procedure where there are few data available, such consultation with conflicting opinions, make a decision as the dorsal rhizotomy in the late 1980s. The mother was that does not turn out well. This decision-making process able to come to terms with feeling badly about her daugh- can inflame the process of coming to terms with the child’s disability further, making the parents feel that they are

20 Cerebral Palsy Management themselves partially to blame. This concept of who is to sion in both the child and family members and marital blame and why this has happened seems to get magnified stress, and may aggravate substance abuse. It is important at adolescence, especially with development of major de- in such families that the family stresses are identified and formities and surgery, such as a posterior spinal fusion. that good psychiatric consultation be obtained for both These issues often lead to family stress, including depres- psychologic and pharmacologic management. A Plan for Managing Complications Discussion of possible complications is also important; however, the ex- pected outcome should be honestly approached. Some surgeons tend to have very pessimistic expectations with regard to expected outcome and compli- cations. Surgeons with this approach soon overwhelm themselves and their families with their assessment of the poor balance between the expected out- come and the possible complications. Most surgeons who have a large CP practice tend more toward the overly optimistic approach in which the out- comes clearly will be worth the risk of the complications. The risk of an overly optimistic approach to families occurs when there are complications. These families may be surprised and angry and find it difficult to deal with the unexpected. It is difficult for physicians to have the perfect balance, but each physician should be aware of their own tendency. Usually, an honest assessment and feedback from partners will identify which personality trait, either optimistic or pessimistic, a physician tends to use when approaching families. By recognizing this tendency, surgeons can be more sensitive to what families are hearing and make suggestions to moderate this perception. There are families who for some reason or another have not been ob- taining appropriate orthopaedic care for their children. Then, when these children are adolescents, they may come to see a CP surgeon with a painful hip dislocation, severe scoliosis, or other deformities that are in a severely neglected state. Some of these families are surprised to hear that only a sur- gical procedure will be the appropriate treatment. Some families may be very resistant to surgery and will want to try everything else. These families must understand that only surgery will correct the problem, but the surgery sel- dom has to occur on an emergency basis. If a surgeon perceives a family’s hesitancy, and attempts to mollify them by suggesting that a brace, injections, or some other modality be tried even though it will provide no long-term benefit, the family will likely hear uncertainty in the physician’s approach. Families may miss the message completely that only surgery will address the problem when they are appeased by nonsurgical treatment. Giving chil- dren temporizing measures to provide relief of pain is appropriate; however, doctors must be clear to families that these measures are only providing tem- porary pain relief and are not treatments. By giving families a little time with the use of these temporary measures, physicians can develop a relationship with the families. There are situations where medical and psychiatric treat- ment may be required before the surgical treatment can occur. For all these reasons, it is important to be clear about the required treatment, its expected outcomes, and then to outline the full treatment plan. As this treatment plan is undertaken, the relationship a physician has developed with children and families will allow them to be confident that the recommended treatment can occur in a safe and effective way.

1. The Child, the Parent, and the Goal 21 When Complications Occur When treatment of a child does not go well, the orthopaedist must first rec- ognize this as a complication. The judgment of recognizing a complication is one of the most difficult to develop and some physicians may never do it well. Many complications, especially in orthopaedics, do not present with the drama of a cardiac arrest. In orthopaedics, a more typical example is the presentation of a deep wound infection. Every wound with a little erythema and a mild superficial drainage is not a deep wound infection. However, when a deep wound infection is present, it should be acknowledged as such. These families should be told of the complication and a definitive treatment plan should be described (Case 1.3). For this process to work, physicians first have to acknowledge the complication to themselves. We have seen many physicians who cannot bring themselves to acknowledge the magnitude of the complication. Likewise, we have seen physicians who overreact to rela- tively minor problems that will resolve if left alone. Finding a balance requires physicians to be honest with themselves and be aware of their own tendency toward optimistic or pessimistic ends of the spectrum. The optimist tends to see the complication as minor variance of normal, whereas the pessimist tends to be overly concerned that any wound change may be a deep wound infection. By being aware of one’s own ten- dency, as experience is gained, an approach to diagnosing and acknowledging complications and then making specific treatment plans will be developed. Complications tend to make physicians feel like failures, and a good retro- spective evaluation of the treatment course may demonstrate errors of judg- ment or execution. These errors should be viewed as learning experiences and opportunities to teach oneself as well as others. A significant number of the case histories in this book are careful analyses of complications that have occurred in our practice. It is important that the approach to analyzing a complication is to determine the exact cause of the complication when possible so that it may be avoided in the future. Saying that “I will never do that operation again” is an inappropriate response to complications. This response comes very close to that of people who say they will never get in a car again after they have had a car accident. Our goal is to always have a complication-free treatment and recovery for every patient; however, we learn the most from careful analysis of our complications and poor outcomes. Once physicians acknowledge the complications to themselves, the fam- ilies then need to be told. Families may react with quiet acceptance, frustra- tion, or anger. These feelings are often the same feelings that physicians have about the same complication. If physicians are willing to share some of their frustration and concern about the complications, it often helps families to put the problem in perspective. It is very important to explain to families what to expect from a complication. This explanation should include a de- tailed outline of the expected treatment plan. If a complication arises that physicians are not comfortable treating, getting a second opinion from, or seeking the help of, another physician is very important. This step should be explained carefully to families. Frequent contact with families is very impor- tant, especially if they develop considerable anger and anxiety, because if they feel that the doctor is trying to avoid them, these feelings often increase. Complications should be managed very much like the initial decision to have an operation. First, specific problems should be carefully defined to fam- ilies. Next, the range of options and expected outcomes, with respect to the short- and long-term implications, should be placed forward as specifically

22 Cerebral Palsy Management as possible. As much as possible, families should be told the detailed expected timeline and exact treatments. For instance, if repeat or additional surgery is expected in the future as a consequence of a complication, this should be laid out for families. If antibiotics are to be used, families should be told for how long and what factors will be monitored to determine a good outcome. This kind of detail gives families a sense that there is someone in charge with experience in dealing with these complications and helps them deal with the fear of the unknown, which the complications often bring to the foreground. Complications need to be recorded in detail in the medical record and should reflect all the objective observations and alternatives that were con- sidered. This record is not the place where blame should be directed. What is observed to have occurred should be documented objectively without re- writing history. For example, if the toes are found to be insensate and with- out blood flow in a child who has had a cast on a foot following surgery, this should be reflected in the medical record, followed by a recording of the immediate action taken, such as removing or opening the cast, and the out- come of that action, such as the improved and returned blood flow to the toes. There is no reason to speculate that the cast was applied too tightly, or that the nursing staff failed to elevate the cast, and so forth. This kind of analysis is important, but should be done after the patient is treated appro- priately and there has been time to reflect on the whole situation. Often, these initial assessments are incomplete and wrong and most frequently are written to protect the writer. Later, during a more thorough investigation or legal action, these assessments only make it appear as if the writer was try- ing to cover up or shift blame to someone else. During stressful treatment periods, especially when dealing with difficult complications, it is very important to ask partners and other colleagues to evaluate the patients and give unbiased opinions. A treating physician can develop a biased view, especially in the face of complications where one would not like to acknowledge personal culpability. Involving other colleagues also gives families the sense that their physician really is trying to keep all options open. If these consultants do have different opinions, these opinions should be discussed between the physicians first, then the options should be outlined for families with a unified recommendation wherever possible. Giving fam- ilies different treatment recommendations and expected treatment outcomes from several different consultants should be avoided. The Final Goal The goal in treating children with CP is for them to grow and develop within the context of a normal family. Their medical treatment and medical condi- tion should be an experience just as a normal part of who they are. For ex- ample, a 6-year-old child who fractures her femur will have a 6-month treat- ment course until most of the rehabilitation is completed. This occurrence will remain a definite event in the child and family’s growth and develop- ment; however, when she is graduating from high school and going off to college, this medical event probably will have faded into many other growing- up experiences. This is the pattern that we want to try to mimic in children with CP (Case 1.5). In the past, children might have spent 30% to 50% of their growing-up years in hospitals having and recovering from surgeries trying to make them walk better or to make them straighter, which was very detrimental. Mercer Rang termed this the “birthday syndrome,” in which children were in the hospital for most of their birthdays, and nurses were baking their birthday

1. The Child, the Parent, and the Goal 23 Case 1.5 Emily Emily was born premature at 28 weeks weighing 1500 g. In her high school years, her crouching gait pattern She was in the hospital for 2 months following birth. increased slightly during the adolescent growth spurt but Following her discharge, she was recognized to have in- then leveled off as her growth completed. On several oc- creased tone in her lower extremities with some develop- casions, we had recommended additional muscle length- mental delay early on. By age 4 years, she was developing ening and realignments to assist her in having a more substantial contractures and had an adductor, hamstring, upright posture. She was always clear that she was not and tendon Achilles lengthening. She was noted to have having any pain with walking, she was doing well walk- rather severe neural deafness. In addition, several eye sur- ing, and she herself was not interested in any more sur- geries were performed in childhood. She started school gery. At the time of these discussions, she would always with some educational support and special treatment for listen carefully to the recommendations. Because she per- the deafness, but was noted to have excellent cognitive ceived herself as doing well, she could see no benefit in functioning. She succeeded in school with assistance of having surgery. special support for her hearing disability. At one point, she was sent to a boarding school specializing in teaching Emily, in spite of having two substantial disabilities, the children with hearing disabilities. However, after 1 year, diplegic pattern CP, and a significant hearing disability, she missed interaction with her family and returned to the was able to have a childhood and adolescent experience normal school setting. very similar to her age-matched peers. She is an excellent example of success in reaching our real goal of treatment, She had two additional medical treatments, one at because she has responded to many of the stresses of age 10 years for additional muscle lengthening, and one growth and development similar to her age-matched peers, at age 13, which consisted of a triple arthrodesis of her even to the point of dropping out of college and deciding feet, hamstring lengthenings distally, and a rectus trans- that she would rather go to work. It is especially signifi- fer at the knee. She continued to walk in the community cant that after she dropped out of college, she has worked with a combination of Lofstrand crutches and a walker. for several years now as a teacher’s aide, a job she greatly Around the house, she would walk holding on to furni- enjoys. She continues to have the goal of returning to col- ture. During her high school years, she developed a mildly lege and becoming a teacher. We are quite confident that increased crouching gait pattern and was placed in a in time she will accomplish this goal because she has a ground reaction ankle foot orthosis (AFO), which she strong sense of who she is and a strong sense of what she disliked. However, she acknowledged that the braces al- wants to do. Most of this has come from an excellent fam- lowed her to walk easier so she would use them for am- ily environment in which she was given strong structure bulation in the community. In high school, she did very but also allowed to express herself. She is an example well both academically and socially. By age 16 years, she of an individual who did not end with the ideal medical was working as a camp counselor for children with hear- treatment because the crouched gait pattern she currently ing disabilities during the summer; at age 18 years she has as a young adult could probably be improved; how- obtained a driver’s license. At age 18, following gradua- ever, it has been her choice to not pursue further treat- tion from high school, she entered college. Her goal on ment. The positive assessment we can make as physicians entering college was to become a teacher; however, after is that the medical care that was provided has not inter- a little over 1 year in college, she became tired of the col- fered with her growth and development as a competent lege scene and was interested in going to work and being functioning adult. closer to her family and community. cakes and having birthday parties for them rather than their families at home.1 Many of these children came to see the hospital staff as a second family (Fig- ure 1.2). This seldom happens currently because of greatly shortened hos- pital stays and improved diagnostic abilities. For most children with CP, all orthopaedic management should ideally be done with only two major sur- gical events during their growth and development. This ideal is not possible

24 Cerebral Palsy Management Figure 1.2. The typical approach to the sur- gical treatment of children with CP was to perform a surgery almost every year. This con- cept often led to children spending a great deal of time in the hospital, to the point where the nursing staff would become “pseudo- parents,” more often celebrating birthdays with the children than the children’s own families. to achieve in all children but should continue to be the goal. Striving for de- creasing the number of orthopaedic operative events in children’s lives and moderating the amount of other medical treatments to only those that will have definite and lasting benefit should be continued. For example, an am- bulatory child with normal cognitive function should not be having physical or occupational therapy at any time that interferes with their education. Therapeutic goals should be planned during summer months or in ways that do not interfere with education. Twenty years ago, the use of inhibition casting was popular. It was be- lieved that this technique decreased contractures and managed spasticity. These children were in leg casts for 8 weeks, often requiring trips to the clinic to change the cast every 2 weeks. After 2 or 3 months, the whole process would have to be repeated. If families could tolerate the stress, although few did, these children would be in a cast for 30% to 50% of their growing years. The time and behavioral stress placed on these families meant that a large part of their lives revolved around their children’s medical treatments. When these children graduated from high school, they tended to see all these cast- ing events as a major focus of their growing-up experience instead of the more normal childhood growing experiences, such as going to the beach, going to Disney World, or other parties and events. In young adulthood, the success of the whole individual with CP is de- termined much more by the family and the individual’s educational experi- ence than by the activities of the medical treatment. The medical care system can help children and families cope with the disability and allow individuals with CP to function at their maximum ability. However, the medical care sys- tem also must recognize that too much focus on perfection of function may cause damage to the growth and development of the children and family unit, especially in the social, psychologic, and educational domains. Achieving this balance varies with each child and family. For example, many successful young adults without disabilities do not have the ideal maximization of their physical function because the focus of their interests is sedentary activities. Just as with these nondisabled young adults, there is great variation in how important maximizing physical function and appearance is to each individ-

1. The Child, the Parent, and the Goal 25 ual with CP. When young adults are truly able to make informed and well- articulated decisions, then they have arrived at a level of success in young adulthood. Just as with nondisabled adolescents and young adults, the med- ical care providers should stress the importance of good physical condition- ing; however, trying to enforce a specific level of physical activity against the person’s wishes tends not to be very productive. Individuals with disabilities should be allowed to make these decisions in the same way that individuals without disabilities are allowed to decide, even if their physician thinks it is not in their best interest. Therefore, the final goal is to encourage the devel- opment of individual adults who are as competent as possible to make their own decisions, who develop the confidence to make those decisions, and are then willing to make decisions and live with the consequences. Always in the context of this final goal, we as therapists and physicians want the individ- ual’s physical impairments minimized as much as technically possible. Reference 1. Rang M. Cerebral palsy. In: Morrissy R, ed. Lovell and Winter’s Pediatric Ortho- pedics, Vol. 1. Philadelphia: Lippincott, 1990:465–506.

2 Etiology, Epidemiology, Pathology, and Diagnosis Cerebral palsy (CP) is a static lesion occurring in the immature brain that leaves children with a permanent motor impairment. The lesion may occur as a developmental defect, such as lissencephaly; as an infarction, such as a middle cerebral artery occlusion in a neonate; or as trauma during or after delivery. Because brain pathology in all these etiologies is static, it is consid- ered CP. Many minor static lesions leave no motor impairment and do not cause CP. Many pathologies, such as Rett syndrome, are progressive in child- hood, but then become static at or after adolescence. These conditions are not part of the CP group, but after they become static, they have problems very similar to those of CP from the motor perspective. Other problems, such as progressive encephalopathy, have very different considerations from the motor perspective. Saying a child has CP only means the child has a motor impairment from a static brain lesion, but says nothing about the etiology of this impairment. Some authors advocate using a plural term of “cerebral palsies” to imply that there are many kinds of CP.1 There is some validity to this concept, similar to the term “cancer,” in which many specific pathologic types of cancer, each with a different treatment, are recognized. Although applying this concept to CP is appealing from the perspective of determining etiologies and under- standing the epidemiology, it provides very little help in actually managing the motor impairment. From the cancer analogy, for example, the specific cellular type and stage of breast cancer are important to know to prescribe the correct treatment. With CP, knowing the cause does not help treat a child who has a dislocated hip. The treatment is based on the diagnosis of CP, as opposed to a muscle disease, spinal paralysis, or a progressive encephalo- pathy. The original cause of the CP does not matter. Therefore, the concept of “cerebral palsies” is not used in the remainder of this text, and the term cerebral palsy will not carry any information on specific etiology. Although the etiologic information has little relevance in the management of motor impairments, it is of limited importance in some children for giving a prog- nosis. The etiology can be important to families in terms of genetic counsel- ing with respect to the risks of future pregnancies, and it is important as an outcome measure for nurseries and epidemiology. Physicians who manage the motor impairments must always maintain a healthy suspicion of the diagnosis of CP, as sometimes a dual diagnosis may be present or the original diagnosis may be wrong. When progression of the impairments and disability, along with a child’s maturity, do not fit the usual pattern of CP, more workup is indicated. For example, a child may be diag- nosed with diplegia because he was premature and had an intraventricular hemorrhage, but, by age 6 years, the physical examination demonstrated very

28 Cerebral Palsy Management large calves with much more weakness and less spasticity than would usually be expected. This child would need to be worked up for muscle disease with the understanding that he can have both Duchenne’s muscular dystrophy and diplegic pattern CP. Alternatively, the child’s history may have been a red her- ring and he does not have CP, but does have Duchenne’s muscular dystrophy. There are children born prematurely who have intraventricular hemorrhages but are completely normal from a motor perspective. Etiology of Cerebral Palsy As noted previously, there are many causes of CP, and knowing the exact etiology is not very important for a physician managing the motor impair- ments. The etiology may be important when considering whether a child is following an expected course of maturation and development. Also, parents find the etiology important because it is part of coming to terms with the larger question of why the CP happened. Many etiologies can be separated into a time period as to when these insults occurred. For more detailed in- formation on the etiologies of CP, readers are referred to the book The Cere- bral Palsies by Miller and Clarke,1 which provides much greater detail on this specific topic. Congenital Etiologies A whole group of congenital developmental deformities lead to CP. These deformities result from defects that occur in normal development and follow patterns based on failures of normal formation (Figure 2.1). A defect of the neural tube closure is the earliest recognized deformity leading to survival with motor defects. The most common neural tube defect occurs in the spine and is known as meningomyelocele. However, this lesion typically does not cause CP, but instead causes spinal-level paralysis. In the brain, the neural tube defect is called an encephalocele, and may be anterior, with a major mid- face or nasal defect. Anterior encephaloceles occurs most commonly in Asia, whereas posterior encephaloceles most often occur in Western Europe and America and affect the posterior occiput.1 The cause of this regional differ- ence is unknown; however, just as folate used during pregnancy has been found to protect against myelomeningocele development, it is believed to pro- tect against the development of encephalocele as well.1–3 Some encephalo- celes are related to larger syndromes, such as Meckel’s syndrome.4 This syndrome includes encephalocele with microcephaly, renal dysplasia, and polydactyly and is due to a defect on the 17th chromosome, specifically in the homeobox gene (HOX B6). This information suggests that many of these deformities may have unrecognized genetic causes. Most children with sig- nificant encephaloceles have very significant motor impairments, usually quadriplegic pattern involvement with more hypotonia than hypertonia. Segmental defects in the brain are called schizencephaly, meaning there is a cleft in the brain.5 These schizencephalies vary greatly, from causing minimal disability to causing very severe quadriplegic pattern involvement, usually with spasticity and mental retardation. Several patients with severe forms have genetic defects in the homeobox genes. Primary proliferation defects of the brain lead to microencephaly. How- ever, there are many causes of microencephaly, most involving toxins or infections, which are discussed later. Conditions in which the brain is too large are called megaloencephaly, which should not be confused with macro- encephaly, meaning a head that is too large. Megaloencephaly is caused by

2. Etiology, Epidemiology, Pathology, and Diagnosis 29 A Figure 2.1. In the earliest stage, the neural plate differentiates from the ectoderm, then enfolds to create a neural tube. Failure of this enfolding causes neural tube defects (A). Dur- ing the embryonic stage, this neural tube de- velops complex folding with the formation of flexures. During the period of 30 to 100 days of embryonic life, the brain demarcates and develops the cerebral hemispheres. During the rest of gestation, there is a large growth of mass and cell specialization (B). B

30 Cerebral Palsy Management Figure 2.2. As the brain matures, the cells proliferate centrally and migrate toward the cortex. During this migration, trailing con- nections remain to the deep layer. This migra- tion is an important element in the formation of the gyri of the cerebral cortex. Defects in the migration lead to a smooth brain surface called lissencephaly. cellular hyperproliferation, usually in syndromes such as sebaceous nevus syndrome, whereas macrocephaly most often is due to hydrocephalus. During development, the neurons migrate toward the periphery of the brain, and a defect in this migration pattern leads to lissencephaly, meaning a smooth brain, or a child with decreased cerebral gyri. Lissencephaly usu- ally leads to severe spastic quadriplegic pattern involvement, but there is a significant range of involvement. Lissencephaly is X-linked in a few cases. The opposite of too few gyri seen in lissencephaly is polymicrogyria, in which there are too many small gyri (Figure 2.2).1 A large and variable group of children have differing degrees of cortical dysgenesis, which is a disorder of brain cortex formation. This disorder may be called focal cortical dysplasia and presents mainly with seizure disorders. The motor effects may vary from none to very severe and from hypotonia to hypertonia. Another part of normal development of the brain in the neonatal and pre- natal period requires formation of the synapses and then subsequent re- modeling of this neuronal synapse formation. As the cells migrate into the correct position and initially form their synapses, many of these premature synapses need to be remodeled through the influence of external stimuli for normal function to develop. The classic demonstration of this principle was shown in the experiment in which eyes of kittens, one each kitten, were sewn closed at birth. The eye that was denied light stimulation became cortically blind; however, the opposite eye that did get light and normal stimulation became overrepresented in the cortex of the brain.6 This experiment has be- come the basis for treating and understanding amblyopia, or lazy eye, in chil- dren. The synaptic remodeling and formation, also called synaptic plasticity in older ages, continues throughout life and is the basis for much of learn- ing. The nature of this synaptic remodeling potential changes with age as demonstrated by the example with the kittens. If the kitten whose eye was sewn shut is denied light stimulation until a certain age, it can no longer re- cover the ability for sight in that eye.6 This concept of synaptic formation and remodeling has been the basis of some therapy programs, specifically the patterning therapy proposed by Doman and Delacatta.6–8 There is no scientific evidence to suggest that the human gait generator can be accessed and impacted in the same way one can

2. Etiology, Epidemiology, Pathology, and Diagnosis 31 treat lazy eye at an early age in children. However, there is a general under- standing that significant seizure activity in a young child may prevent synap- tic remodeling through excitotoxic injury, which leads to CP. Inappropriate synaptic formation and remodeling, or remodeling alone, has been impli- cated as the major neurologic anatomic pathology in Down syndrome, Rett syndrome, autism, and fragile X syndrome as well as many cases of ataxia, idiopathic spasticity, and mental retardation in which there is no other rec- ognized etiology.1 Neonatal Etiologies Neonatal and prenatal causes of CP are mainly related to prematurity and birthing problems, which lead to various injury patterns. However, the im- mature brain has much more equipotentiality or plasticity, both of which are terms used to define the much greater ability of an uninjured part of the immature brain to assume the function of an injured part. This potential of the immature brain to reassign function makes the response to injury much different than in the mature brain. Prematurity and brain hemorrhages are much better understood since the widespread use of cranial ultrasound, in which the infant brain can be im- aged through the open anterior fontanelle. This image provides an excellent view of the ventricles and the periventricular white matter. This is the area where hemorrhages occur, and major risk factors for developing hemor- rhages are younger gestational age and mechanical ventilation. Bleeding in the ventricle is called intraventricular hemorrhage (IVH), and bleeding in the periventricular area is called germinal matrix hemorrhage (GMH), or it may be combined in a term called periventricular-intraventricular hemorrhage (PIVH). A common grading system for the severity of these hemorrhage pat- terns includes grade I with germinal matrix hemorrhage only, grade II with hemorrhage in the lateral ventricle and dilation of the lateral ventricle, grade III with ventricular system enlargement, and grade IV with periventricular hemorrhage and infarctions (Figure 2.3). Reported prognostic significance of these grades varies greatly, and the general consensus is that premature in- fants with no PIVH have a better survival prognosis than those with PIVH.1 Also, in group studies, the more severe the grade, the higher the risk of de- veloping CP, as demonstrated in a study that reported the risk of CP was 9% in grade I, 11% in grade II, 36% in grade III, and 76% in grade IV.9 How- ever, different studies vary significantly, so good consensus values are not currently available. These cerebral hemorrhages evolve from GMH and IVH, which develop in the first 72 hours after birth. The brain bleeds then resolve, and peri- ventricular leukomalacia (PVL) develops 1 to 3 weeks after birth in some children. Periventricular leukomalacia in the form of periventricular echo- genicity (PVE) may be seen on ultrasound, but does not develop cysts. If cysts develop, it is called cystic periventricular leukomalacia (PVC). In general, infants with PVC have the highest risk of developing CP and infants with PVE have the lowest risk.10 In one study, 10% of children developed CP if they had PVE; however, 65% developed CP if they had PVC.9 Again, these numbers vary between studies. The general trend is that premature infants with more severe bleeds have a worse prognosis for survival and a higher risk for developing CP; however, there are no specific parameters that fully predict risk of developing CP or, much less, predict the severity of CP in an individual child. Hypoxic events occurring around delivery, usually in full-term infants, also lead to disability. These events have been termed hypoxic-ischemic

32 Cerebral Palsy Management Figure 2.3. Bleeding in the immature brain occurs primarily around the ventricles, which have many fragile vessels. Intraventricular hemorrhage (IVH) means bleeding into the ventricles. Germinal matrix hemorrhage (GMH) means bleeding into the tissue around the ventricles. Periventricular intraventricular hemorrhage (PIVH) means bleeding into both areas. Periventricular cysts (PVC) form in these same areas as the acute hemorrhage resolves. encephalopathy (HIE). The causes of this hypoxia may vary from obstetric dystocias to other anoxic and low-flow states in the neonate. In severe cases of HIE, subcortical cyst formation develops and is called multicystic en- cephalomalacia. In general, when this cystic pattern forms, the prognosis for good function is poor, with most of these children developing severe quad- riplegic pattern involvement with severe mental retardation. Some of these children develop cysts in the thalamus and basal ganglia, which may lead to dystonia.1 Neonatal stroke occurring in the preterm or full-term infant usually in- volves the middle cerebral artery and presents as a wedge-shaped defect in one hemisphere. These defects may develop as cysts, which, if very large, are called porencephaly or porencephalic cysts. In general, if these wedge-shaped defects are small, the children may be normal; however, a significant defect especially with a cyst usually presents as hemiplegic pattern CP. Even with large cysts, these children’s function, especially cognitive function, may be quite good. Postnatal Causes of Cerebral Palsy Postnatal causes of CP may overlap somewhat with the prenatal and neonatal group; however, postnatal trauma, metabolic encephalopathy, infections, and toxicities are considered as etiologies in this group. Although the data are difficult to assimilate, between 10% and 25% of CP cases have a post- natal cause.11,12 Child abuse or nonaccidental trauma causing brain injury in a young child may be due to blunt trauma with skull fractures or fall into the pattern of shaken baby syndrome. Shaken baby syndrome occurs usually in a child less than 1 year of age when a caretaker shakes the baby back and forth to quiet the crying. This vigorous shaking causes stretching, shearing, and tear-

2. Etiology, Epidemiology, Pathology, and Diagnosis 33 Figure 2.4. Shaken baby syndrome creates an injury in which axons are disrupted by the shear forces created from the violent shaking of the head. The brain of the baby is like an egg in which the liquid center is enclosed in a solid outer shell. By vigorous shaking, the egg yolk can be broken without breaking the shell of the egg. In the same way, vigorous shaking of a baby’s head can cause tissue dis- ruption. This shearing stress disrupts brain tissue, especially the long migrating axons of the cerebral cortex. The trauma of the shaken baby does not usually cause a skull fracture and may not even cause intracranial bleed- ing, but it often causes severe long-term neu- rologic impairment because of the cellular disruptions. ing of the long axons and capillaries in the cortex of the brain (Figure 2.4). If these babies survive, they often have a severe spastic quadriplegic pattern involvement with a poor prognosis for improvement.5 Even children with less severe motor involvement often have a concomitant profound mental retardation. Blunt head trauma may also occur from child abuse, falls, or motor ve- hicle accidents, and it involves the direct injury as well as the secondary in- jury from brain swelling. Most children with blunt trauma recover and have no motor defects.13 However, if there is a unilateral bleed, these children are often left with a hemiplegic pattern motor disability. The more severely in- volved children are usually left with a severe quadriplegic pattern involve- ment and do not become functional community ambulators. Many children with motor impairments from closed head injuries have ataxia as a major impairment. Children with closed head injuries will make substantial improvement for 1 year after the injury and only in rare severe cases should surgical treat- ment of secondary problems, such as contractures, be considered during this year. Also, many children continue to improve even through the third year after injury; therefore, it is probably best not to consider the lesion static until 3 years after the injury.14 Even then, these lesions continue to evolve in some individuals, with the well-recognized syndrome in which early spastic- ity resolves but then dystonic movements later develop in the previously spastic limb. This syndrome has been reported to occur up to 9 years after closed head injury, even when it seemed that all the spasticity had resolved.15 We have seen recurrent dystonia become most severe during and after pu- berty, as the hormonal surge somehow makes it worse. Metabolic encephalopathy has a wide variety of causes, most extremely rare. It is impossible to give a comprehensive review in this text, and when specific cases are encountered, it is important to obtain disease-specific up- to-date recommendations from the subspecialized expert who is managing the care of the child. Also, the neuro-orthopaedist should have a good ref- erence text available, such as the Aicardi text Diseases of the Nervous Sys- tem in Childhood.16 The metabolic disorders can be divided into storage disorders, intermedullary metabolism disorders, metallic metabolism, and miscellaneous disorders (Table 2.1). It is extremely important for physicians caring for children’s motor prob- lems to understand the expected course of the disease. For example, many of the storage disorders are progressive and these children have limited life expectancy, which limits attempts to correct motor impairments that are not

Table 2.1. Metabolic neurologic diseases. Name Primary defect Typical course Significance for Storage diseases surgical management Gangliosidoses intercellular accumulation Most of these have no treatment Hexosaminidase defect, multiple and are progressive Most patients have Tay–Sachs disease types Each type has its own course hepatosplenomegly Sandhoff’s disease HexA and HexB nonfunctional Be especially aware of significant GM1 gangliosidosis due to chromosome 15 defect Short-term survival in childhood splenomegly Gaucher’s disease Type O gangliosidosis Also, bone lesion from the Multiple subtypes, beta- Clinically like Tay–Sachs storage disease may be present galactosidase deficiency Rare cases and variable effects Bone marrow may be involved, Multiple types, beta- and some patients develop a glucocerebrosidase deficient Outcome is variable, based on peripheral neuropathy the subtype, from rapid course Niemann–Pick disease Sphingomyelinase deficient, with death in early childhood to Death is usually from cardiac or multiple subtypes relatively mild involvement renal failure Females are less affected Fabry’s disease Sex-linked deficiency of ceramide The more severe types have rapid May begin as severe muscle pain trihexoside degeneration and death; some Renal failure may occur mild types may have minimal Cerebroside sulfatase deficiency, involvement and life into middle May present with slow-onset Metachromatic leukodystrophy multiple types adulthood hemiplegia or diplegia Foam cells with vacuolated Bone marrow transplantation is Krabbe’s disease (globoid cell Beta-galactocerebrosidase cytoplasm develop in muscles, used to treat a number of these leukodystrophy) deficiency nervous system, kidneys conditions Mucopolysaccharidosis All have deficiencies of lysomal Severe dwarfism glucosidase or sulfatase Often presents as a gait disorder Cervical instability Hurler’s syndrome in childhood Hydrocephalus may develop — May initially look like a Scheie’s syndrome neuropathy Mild to moderate neurologic Hunter’s syndrome — Adult forms present as behavior involvement Sanfilippo’s syndrome problems Minimal skeletal problems Morquio’s syndrome — Age of onset, and survival, are Maroteaux–Lamy’s syndrome variable Cervical instability may cause — Often the neurologic problems spinal cord compression are less severe than the systemic Nerve entrapment syndromes are — ones common Severe neurologic retardation Mild to severe bone and — neurologic involvement Types, very mild to minimal Mild to severe bone and Sly’s syndrome — problems neurologic involvement — Severe dwarfism Mucolipidosis, sialidosis, Late onset glycoprotein metabolism Also called cherry red spot Severe progressive neurologic Has a pure intention myoclonus deficiency myoclonus syndrome involvement that slowly gets worse with age Variable forms but marker bone Sialidosis type one involvement May develop dystonia No neurologic involvement — Severe dwarfism Mucolipidosis IV Very variable Many types, all very rare Slow progression No other involvement Failing vision and mental delay after normal infancy

2. Etiology, Epidemiology, Pathology, and Diagnosis 35 Table 2.1. Continued. Primary defect Typical course Significance for Name Alpha-mannosidase deficiency surgical management Fucosidase deficiency Several types, usually with Mannosidosis Neuraminidase and beta- cognitive limits and minimal Develop significant spasticity Fucosidosis galactosidase deficiency progression Thoracolumbar spinal deformity Galactosialidosis Sialic acid transport deficiency Progressive mental retardation may be present Salla disease Develops progressive myoclonus Course varies Aspartylglycoaminuria Neuronal ceroid-lipofuscinosis and extrapyramidal signs Pompe’s disease Mental and motor retardation, Causes bone deformities, mitral Batten disease (infantile form) Many causes, only those more progressive valve insufficiency relevant included Has mental deterioration in late Severe mental retardation Spielmeyer–Vogt–Sjogren A defect in the hydroxylation of childhood or adolescence Early death (juvenile form) phenylalanine to tyrosine; the Hypotonia Anxiety and autistic behavior Kufs’ disease (adult form) defect may occur in one of two Death after a prolonged Amino acid metabolism enzymes or two required Severe brain atrophy vegetative state Phenylketonuria (PKU) cofactors Has repetitive hand movements Same as PKU Condition starts in middle that may be confused with Rett Hyperphenylalaninemia (HPA) Organic aciduria; many subtypes childhood syndrome Maple syrup urine disease Present with behavioral changes Slower course Glutaryl-CoA dehydrogenase and dementia Death in 15–30 years Glutaric aciduria deficiency Untreated children develop severe With early dietary treatment mental retardation and self-abuse most of the symptoms can be avoided Requires treatment until age 4–8 years Disease varies from rapid May cause acute coma progression to later onset or Treatment varies by the specific minimal progression defect Most of these conditions cause Several types most of the problems during periods of stress when the body may depend on protein metabo- lism for energy source; this is especially true during major sur- gical procedures and can usually be avoided by using high-glucose infusion such as a 10% glucose solution intra- and postoperatively Blood pH level needs to be monitored and urine should be monitored for ketosis If proper precuations are not taken, ketoacidosis, hyper- ammonemia, and hyperlacticemia may develop and cause cerebral edema with further neurologic injury Untreated neurologic effects leave the child with severe dystonia Cognitive process more preserved Stress causes a ketoacidosis, which causes brain injury Neurologic effects can be avoided with early dietary treatment Must take all the same precautions as noted for maple syrup urine disease (continued )

36 Cerebral Palsy Management Table 2.1. Continued. Primary defect Typical course Significance for surgical management Name Cystathionine beta-synthase Cause mental retardation and Homocystinuria deficiency spasticity Develop dislocated lens Also have thromboembolic Sulfite oxidase deficiency During infancy children have disorder poor feeding, severe seizures, and May present with a Charlie Tyrosinemia present with quadriplegic pattern Chaplin-like walk motor involvement Other common bone deformities Tetrahydrobiopterin Same pathway as PKU and HPA Usually die in early childhood include pectus, genu valgum, Present with liver failure and biconcave vertebra, deficiencies (“malignant HPA”) neuropathy epimetaphyseal widening Because of the thromboembolic Nonketotic hyperglycinemia Glycine accumulates because it Children have progressive problems, even children should cannot be metabolized deterioration even with probably have anticoagulation appropriate dietary treatment during surgical procedures 4-Hydroxybutyric aciduria GABA neurotransmitter Children have progressive Urea cycle disorders metabolism error spasticity and limb rigidity Also often complain of severe leg Sometimes with dystonia or pain Ammonia accumulation causes athetosis Course is variable brain injury Course is usually with severe Clinical course is variable seizures and short-term survival, although some develop a more These conditions are like maple typical spastic CP pattern syrup urine disease in that during Presents with a static hypotonia stress periods, such as acute and ataxia sepsis or major surgical There are a number of different procedures, patients must be deficiencies, all with a similar protected from high protein presentation, but with varying metabolism, which will cause the severity ammonia level to raise, running the risk of developing cerebral Citrullinemia edema; this can be prevented Argininosuccinic aciduria with high-glucose fluid infusion, usually using 10% dextrose Arginase deficiency Hepatomegly common Often have brittle hair Hepatomegly common Usually presents as a quadriplegic pattern CP with progressive spasticity Vitamin metabolism disorders Many are autosomal dominant Skin rash, hypotonia, seizures, Symptoms improve with high- inherited ataxia dose biotin treatment Multiple carboxylase deficiency Impairment of the biotin Anemia, seizures, mirocephaly, Vitamin B12 metabolism defect recycling pathway pancytopenia, malabsorption Variable presentation Folate metabolism defect Similar to B12 deficiency

2. Etiology, Epidemiology, Pathology, and Diagnosis 37 Table 2.1. Continued. Primary defect Typical course Significance for Name surgical management Lactic acidosis (respiratory chain Defect in the terminal step of the disorders) energy production cycle The workup and diagnosis of many of these conditions require Mitochondrial cytopathy Usually presents in early infancy a skeletal muscle biopsy because or early childhood with delayed the muscle is often involved Multisystem disorders motor skills, fatigue, muscle This biopsy is also how to study Kearns–Sayre syndrome pains mitochondrial function Mitochondrial myopathy The response is variable, from Normal at birth long static period to spontaneous Alpers syndrome improvement to sudden Ragged red muscle fibers Often present with stroke-like deterioration Leigh syndrome symptoms between childhood and young adulthood Develop headaches, mental retar- Lactic acidosis dation, peripheral neuropathy Pyruvate dehydrogenase Many different defects are Autosomal recessive condition of High incidence of heart block deficiency probably causing this clinical progressive spastic quadriplegic and, if surgery is planned, the Mitochondrial fatty acid syndrome pattern CP syndrome team needs to be prepared to defects insert a cardiac pacemaker Carnitine deficiency Syndrome defined by necrotizing Course is extremely variable but encephalomyelopathy usually progressive, although Some die in early childhood and Peroxisomal disorders Probably has multiple molecular there may be long static periods others survive long term with a Zellweger syndrome causes severe quadriplegic CP pattern Adrenoleukodystrophy Defect of pyruvate entry to Presents with highly variable Under stress, such as major Refsum’s disease mitochondria hypotonia, seizures, failure to surgery, must give high-glucose X-linked thrive infusion or there will be no adrenoleukodystrophy Because of inability to metabolize energy even for the heart to Rhizomelic chondrodysplasia protein, depends on glucose for Very variable with muscle function punctata energy weakness, cardiomyopathy, Wilson disease seizures Poor swallowing Failure to thrive Lesch–Nyhan syndrome Presents in childhood with Develop severe equinovarus feet Enzyme defect allowing muscle weakness and and flexion contractures cardiomyopathy Stippled calcification in the bones, especially the patella All have autosomal recessive inheritance Calcification in the epiphysis and soft tissues Hypotonia Also with mental retardation Later develop a Parkinson-like Disorder of copper metabolism Same as Zellweger but milder presentation with psychiatric form problems Similar but is the mildest form Have hepatic dysfunction Variable, but males are always When giving medication, must more affected than females consider liver function Rhizomelic dwarf with joint Develop gouty arthritis contractures Early on have facial masking, then develop tremor X-linked Very variable course and usually presents with hypotonia, torsional dystonia, mental retardation, self-abuse

38 Cerebral Palsy Management seriously disabling. Alternately, many disorders of intermedullary metabolism have acute insults during toxic events before the diagnosis has been made. With proper management, these disorders become static and mimic similar children with CP. These metabolic disorders often require very specific management pro- tocols during surgery. An example of such a condition is glutaric aciduria type 1, which presents with infants who are normal. When an infant expe- riences a stress, such as a childhood illness with a high fever, an acidosis de- velops that causes damage to the brain, especially the putamen and caudate areas. This insult leaves the child with a wide range of spastic and movement disorders, often with significant dystonia.17 This neurologic disorder is static if the proper dietary management is carried out; therefore, the orthopaedist can approach this child similarly to a child with CP. However, these children must be prevented from becoming acidotic during operative procedures by infusing high levels of glucose, usually using a 10% dextrose solution as the intravenous fluid. A wide variety of infections leave children with permanent neurologic deficits. Most of these deficits are static and therefore definitely fall into the CP diagnosis group. Prenatal and neonatal viral infections are the most com- mon infectious cause of CP. Cytomegalovirus (CMV) leaves 90% of children with mental retardation and deafness, but only 50% develop CP or motor defects. Children who develop congenital rubella infections very commonly will have mental retardation; however, only 15% develop CP.1 Neonatal her- pes simplex infection has a high mortality rate, and 30% to 60% of survivors have some neurologic sequelae, although CP is not common. In utero vari- cella zoster infection causes high rates of CP. This same high rate is seen in lymphacytic choriomeningitis, which is a rodent-borne arenavirus. All these conditions cause neurologic insults that are static and should be treated as CP. Infections with human immunodeficiency virus (HIV) may cause neuro- logic sequelae; however, this is a progressive encephalopathy and these chil- dren should be treated anticipating a very short life expectancy. The most common parasite is Toxoplasma gondii, which is an intracellular parasite whose most common host is the household cat. With aggressive medical treatment, the infection can be eradicated, and approximately 30% of chil- dren are left with CP and mental retardation. Neonatal bacterial meningitis may be caused by many organisms and may be very severe, with as many as 30% to 50% of survivors having CP.1 In our experience, most of these chil- dren who survive bacterial meningitis and have CP will have very severe spastic quadriplegic pattern involvement. Temporary neurologic deficits are caused by many toxic agents, with al- cohol being the most commonly encountered. Alcohol almost never causes a static neurologic deficit. Also, children with prolonged anoxic events, such as near drowning, near hanging, or near asphyxia, can make remarkable re- coveries. However, when these children do not recover completely, they are usually left with extremely severe neurologic deficits and are among the most neurologically disabled individuals in our practice. These children tend to be relatively healthy and, in spite of severe neurologic deficits, tend to grow and thrive physically with good nursing care. One child in our practice has been ventilator dependent for 10 years from an anoxic event at age 9 months. As noted in the beginning of this chapter, knowing the exact etiology is not always important to care for children’s motor disabilities; however, it is important to understand whether these lesions are static or not. Also, par- ents may be more relaxed if physicians and therapists have some under- standing of the specific etiology, if known, of their children’s problems.

2. Etiology, Epidemiology, Pathology, and Diagnosis 39 Epidemiology Because of the wide variety of causes of CP, the exact numbers from differ- ent studies do not completely agree. However, there is remarkable similarity in the prevalence across the world, from Sweden in the 1980s with a preva- lence of 2.4 per 100018 and 2.5 per 1000 in the early 1990s,19 2.3 per 1000 from Atlanta,11 and 1.6 per 1000 in China.20 Considering the difficulty in making specific diagnoses, and especially finding mild cases, these numbers probably reflect much more variation in counting than clear differences in prevalence. A report from England, which is representative of many studies, shows that there has not been much change in prevalence over the past 40 years. However, the patterns of CP have shifted more toward diplegia and spastic quadriplegia and away from hemiplegia and athetosis.21 This change probably reflects increased medical care with better obstetric care and some increased incidence from survivors of neonatal intensive care units. Also, multiple births have increased with increasing maternal age,22 and these multiple births have a substantially higher risk of developing CP. The re- ported prevalence rate per pregnancy for singles is 0.2%, for twins 1.5%, for triplets 8.0%, and for quadruplets 43%.23 Terminology and Classification Although understanding the specific etiology of CP is not very helpful for physicians treating motor problems, by segmenting this very diverse con- dition by cause, patterns that are useful in planning treatment can be iden- tified. There are many ways of classifying CP, one of which is by etiology. However, for the treatment of motor disabilities it is much more important to classify children by anatomic pattern and specific neuromotor impair- ments than by the cause of the CP. Classifying CP in this way provides a framework in which to discuss the functional problems of individuals in their whole environment. A framework for understanding individuals with limited motor function has been agreed to at an international forum held in 1980, organized by the World Health Organization (WHO). The report is entitled “Classification of Impairments, Disabilities and Handicaps.”24 In this report, the term “im- pairment” defines the primary lesion and pathology, such as the problem with the brain that caused the spasticity, and includes the direct effects of the spasticity, such as the dislocated hip caused by the spastic muscles. “Dis- ability” is used to mean the loss of function that individuals experience be- cause of the impairment; therefore, the inability to walk or sit well is a dis- ability arising from the impairment. The “handicap” is the result of limits in the environment and society, which limit individuals as a result of their specific disability. Therefore, an individual who uses a wheelchair has a handicap if he wants to visit a friend and the only way into the house is up a long flight of stairs. This inability to socialize is the handicap and, for many adults, is what impedes them from being integrated into full society of jobs, friends, and social entertainment. In 1993, the National Center for Medical Rehabilitation Research (NCMRR) added to the WHO classification by dividing impairments into “pathophysiology” and “impairment.” In this classification, “pathophysiol- ogy” refers to the primary problem, such as the brain lesion, and “impair- ment” refers to the secondary effects, such as spasticity and the dislocated hip. “Functional impairment” was added to reflect the inability to do activities

40 Cerebral Palsy Management Figure 2.5. The WHO initially developed a model for disability that was later expanded by the USA National Center for Medical Re- habilitation Research. The concepts of both models are similar, with a focus that expands the understanding that problems of function are related beyond the isolated anatomic prob- lem of an individual person. such as walking that is a direct result of the impairment. “Disability” has retained almost its original meaning, and “handicap” has been renamed “societal limitations” to clarify where the problem of the limitation arises.25 Although there are some merits to the changes NCMRR made to the WHO report for research purposes, the complexity does not work well in thought of daily practice; therefore, in the remainder of this text, the WHO defini- tions and terminology are used (Figure 2.5). Anatomic Classification The most useful primary classification for children with CP is based on the anatomic pattern of involvement. This involvement is the first classification used by physicians treating motor impairments, as it gives a very general sense of severity and a general overview of what patients’ problems likely are. Classification into hemiplegia, which involves one half of the body; diplegia, which involves primarily the lower extremities with mild upper ex- tremity involvement; and quadriplegia, which involves all four limbs, is most useful. In general, individuals with hemiplegia and diplegia can walk, and those with quadriplegia use wheelchairs as their primary mobility device. For patients who do not clearly fit these patterns, many other names have been suggested. Double hemiplegia has been suggested for children with upper and lower extremity involvement that is much more severe on one side than the other. Triplegia has been suggested for individuals who have a hemiplegic pattern on one side and a diplegic pattern in the lower extremities. There are rare children who appear to have hemiplegia and diplegia, which would make anatomic sense, so this term triplegia has some merit; however, it does not aid in treatment planning.

2. Etiology, Epidemiology, Pathology, and Diagnosis 41 Monoplegia is used when one limb is primarily involved; however, from a motor treatment perspective, these children are treated as if they had mild hemiplegia. In North America, the term paraplegia implies a pure lower ex- tremity paralysis and is used only for spinal cord paralysis because almost all children with brain origin disability will also have some upper extremity involvement, although it may be very minor. Pentiplegia is occasionally used to define the most severely impaired individuals who have no independent head control. This term adds little over the use of quadriplegia in planning motor impairment treatment; therefore, it has not gained widespread use. Evolutionary Pathology Even though there are many causes of CP, there are few recurring anatomic patterns of involvement because damage to specific areas, regardless of how the damage occurs, creates similar patterns of impairment. However, a spe- cific region of brain injury can cause variation in the impairments because the initial injury also overlies normal development, which continues after the injury. Because all these injuries occur in the young and immature brain, growth and development over time affects the impairment. A brain injury occurring in early pregnancy, meaning most congenital syndromes, has a dif- ferent presentation than an injury occurring in a 4-year-old child. The first aspect of this pathology is to understand the presence of very early primitive reflexes that should disappear as normal children grow. The cutaneous reflexes, mainly finger and toe grasp, occur with stroking of the skin on the palm or on the sole. The sucking and rooting reflexes are simi- larly initiated with stroking of the face and lips (Figure 2.6). The labyrinthine reflex is a response to the inner ear being stimulated by changing a child’s position (Figure 2.7). When held prone, a child will flex, and when placed supine, a child will extend. The proprioceptive reflexes are initiated by stim- ulating the stretch receptors in the muscles and the position sensors in the joints. This reflex creates the asymmetric tonic neck reflex (ATNR) such that when the head is turned to one side, the leg and arm on that side extend (Fig- ure 2.8). The symmetric tonic neck reflex (STNR) causes the arms to flex and Figure 2.6. The most primitive reflex is the sucking reflex, which is stimulated by contact of the infant’s perioral area (A). The hand (B) and toe grip (C) grasp reflexes are also pres- ent at birth and are stimulated by stroking the palm or plantar surfaces. Babies’ early lives are dependent on the sucking reflex and, before high-level medical care, babies who lacked the sucking reflex always died.